Trial Outcomes & Findings for Research Study to Look at How Well the Drug Concizumab Works in Your Body if You Have Haemophilia Without Inhibitors (NCT NCT04082429)

Last Updated: 2025-12-05

Results Overview

Rate of treated spontaneous and traumatic bleeding episodes for haemophilia A participants without inhibitors is presented. The observation period used for reporting this endpoint is on-treatment without ancillary therapy excluding data before restart (OTwoATexBR). It is defined as the time period after the restart where participants are treated by concizumab treatment or are treated by on-demand treatment and additionally have not used factor-containing products not related to treatment of a bleeding episode. The data is reported in the terms of annualised bleeding rate (ABR). Week 0 is defined as time of randomisation to on-demand administration after the pause or time of start of the new concizumab dosing regimen. Confirmatory analyses cut-off was defined as when all participants on no PPX (arm 1) had completed the 24-week visit or withdrawn and all participants on concizumab PPX (in arms 2 and 4) had completed the 32-week visit or withdrawn.

Recruitment status

ACTIVE_NOT_RECRUITING

Study phase

PHASE3

Target enrollment

156 participants

Primary outcome timeframe

On demand (arm 1): From week 0 up until start of concizumab treatment (week 24); Concizumab (arm 2): From week 0 up until the confirmatory analyses cut-off

Results posted on

2025-12-05

Participant Flow

The trial was conducted at 68 sites in 31 countries.

Data is reported till cutoff date 27-Dec-2022 and study is still ongoing. Initially, participants were randomised to arm 1 or 2 or to non-randomised arms 3 or 4. There was a treatment pause due to investigation of thromboembolic events. After treatment pause, participants randomised to arms 1 or 2 before pause entered arm 4. Participants allocated to arms 3 and 4 before pause re-entered arms initially allocated to. New participants were randomized in arms 1and 2.

Participant milestones

Participant milestones
Measure	Arm 1: Previous On Demand (OnD) Treatment: No Prophylaxis (PPX) - Participants With HA Participants with HA received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Extension Phase Arm 1: Concizumab PPX - Participants With HA Participants with HA who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HA Participants with HA received a loading dose of 1.0 milligrams per kilograms (mg/kg) concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Arm 3: Concizumab Non-naive: Concizumab PPX - Participants With HA Participants with HA received a loading dose (participants entering from the phase 2 trial \[N7415-4255\] (NCT03196297) were not to receive a loading dose) of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg.	Arm 4: Concizumab PPX - Participants With HA Participants with HA previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Arm 1: Previous OnD Treatment: No PPX - Participants With HB Participants with HB received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Extension Phase Arm 1: Concizumab PPX - Participants With HB Participants with HB who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HB Participants with HB received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Arm 4: Concizumab PPX - Participants With HB Participants with HB previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.
Main Part (Before the Treatment Pause) STARTED	1	0	3	10	18	1	0	2	2
Main Part (Before the Treatment Pause) COMPLETED	0	0	2	9	13	1	0	2	2
Main Part (Before the Treatment Pause) NOT COMPLETED	1	0	1	1	5	0	0	0	0
Main Part (After the Treatment Pause) STARTED	9	0	18	9	46	12	0	24	30
Main Part (After the Treatment Pause) COMPLETED	7	0	17	6	45	10	0	23	25
Main Part (After the Treatment Pause) NOT COMPLETED	2	0	1	3	1	2	0	1	5
Extension Part STARTED	0	7	17	6	45	0	10	23	25
Extension Part COMPLETED	0	0	0	0	0	0	0	0	0
Extension Part NOT COMPLETED	0	7	17	6	45	0	10	23	25

Reasons for withdrawal

Reasons for withdrawal
Measure	Arm 1: Previous On Demand (OnD) Treatment: No Prophylaxis (PPX) - Participants With HA Participants with HA received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Extension Phase Arm 1: Concizumab PPX - Participants With HA Participants with HA who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HA Participants with HA received a loading dose of 1.0 milligrams per kilograms (mg/kg) concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Arm 3: Concizumab Non-naive: Concizumab PPX - Participants With HA Participants with HA received a loading dose (participants entering from the phase 2 trial \[N7415-4255\] (NCT03196297) were not to receive a loading dose) of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg.	Arm 4: Concizumab PPX - Participants With HA Participants with HA previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Arm 1: Previous OnD Treatment: No PPX - Participants With HB Participants with HB received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Extension Phase Arm 1: Concizumab PPX - Participants With HB Participants with HB who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HB Participants with HB received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Arm 4: Concizumab PPX - Participants With HB Participants with HB previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.
Main Part (Before the Treatment Pause) Withdrawal by Subject	1	0	1	1	3	0	0	0	0
Main Part (Before the Treatment Pause) Physician Decision	0	0	0	0	2	0	0	0	0
Main Part (After the Treatment Pause) Death	0	0	0	0	1	0	0	0	0
Main Part (After the Treatment Pause) Physician Decision	0	0	0	2	0	1	0	0	0
Main Part (After the Treatment Pause) Withdrawal by Subject	0	0	1	1	0	0	0	1	5
Main Part (After the Treatment Pause) Unspecified reason	2	0	0	0	0	1	0	0	0
Extension Part Ongoing	0	7	17	6	45	0	10	23	25

Baseline Characteristics

Research Study to Look at How Well the Drug Concizumab Works in Your Body if You Have Haemophilia Without Inhibitors

Baseline characteristics by cohort

Baseline characteristics by cohort
Measure	Arm 1: Previous On Demand (OnD) Treatment: No Prophylaxis (PPX) - Participants With HA n=9 Participants Participants with HA received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 1: Previous OnD Treatment: No PPX - Participants With HB n=12 Participants Participants with HB received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HA n=18 Participants Participants with HA received a loading dose of 1.0 milligrams per kilograms (mg/kg) concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HB n=24 Participants Participants with HB received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Arm 3: Concizumab Non-naive: Concizumab PPX - Participants With HA n=9 Participants Participants with HA received a loading dose (participants entering from the phase 2 trial \[N7415-4255\] (NCT03196297) were not to receive a loading dose) of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg.	Arm 4: Concizumab PPX - Participants With HA n=46 Participants Participants with HA previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Arm 4: Concizumab PPX - Participants With HB n=30 Participants Participants with HB previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Total n=148 Participants Total of all reporting groups
Age, Continuous	34.7 Years STANDARD_DEVIATION 21.3 • n=37 Participants	30.4 Years STANDARD_DEVIATION 17.5 • n=37 Participants	30.7 Years STANDARD_DEVIATION 9.6 • n=74 Participants	28.0 Years STANDARD_DEVIATION 12.0 • n=267 Participants	43.7 Years STANDARD_DEVIATION 18.0 • n=272 Participants	30.4 Years STANDARD_DEVIATION 13.4 • n=6 Participants	31.6 Years STANDARD_DEVIATION 13.3 • n=36 Participants	31.4 Years STANDARD_DEVIATION 14.2 • n=18 Participants
Sex: Female, Male Female	0 Participants n=37 Participants	0 Participants n=37 Participants	0 Participants n=74 Participants	0 Participants n=267 Participants	0 Participants n=272 Participants	0 Participants n=6 Participants	0 Participants n=36 Participants	0 Participants n=18 Participants
Sex: Female, Male Male	9 Participants n=37 Participants	12 Participants n=37 Participants	18 Participants n=74 Participants	24 Participants n=267 Participants	9 Participants n=272 Participants	46 Participants n=6 Participants	30 Participants n=36 Participants	148 Participants n=18 Participants
Ethnicity (NIH/OMB) Hispanic or Latino	1 Participants n=37 Participants	1 Participants n=37 Participants	0 Participants n=74 Participants	1 Participants n=267 Participants	2 Participants n=272 Participants	4 Participants n=6 Participants	3 Participants n=36 Participants	12 Participants n=18 Participants
Ethnicity (NIH/OMB) Not Hispanic or Latino	8 Participants n=37 Participants	11 Participants n=37 Participants	18 Participants n=74 Participants	23 Participants n=267 Participants	7 Participants n=272 Participants	41 Participants n=6 Participants	27 Participants n=36 Participants	135 Participants n=18 Participants
Ethnicity (NIH/OMB) Unknown or Not Reported	0 Participants n=37 Participants	0 Participants n=37 Participants	0 Participants n=74 Participants	0 Participants n=267 Participants	0 Participants n=272 Participants	1 Participants n=6 Participants	0 Participants n=36 Participants	1 Participants n=18 Participants
Race (NIH/OMB) American Indian or Alaska Native	0 Participants n=37 Participants	0 Participants n=37 Participants	0 Participants n=74 Participants	0 Participants n=267 Participants	0 Participants n=272 Participants	2 Participants n=6 Participants	1 Participants n=36 Participants	3 Participants n=18 Participants
Race (NIH/OMB) Asian	1 Participants n=37 Participants	6 Participants n=37 Participants	8 Participants n=74 Participants	10 Participants n=267 Participants	3 Participants n=272 Participants	13 Participants n=6 Participants	1 Participants n=36 Participants	42 Participants n=18 Participants
Race (NIH/OMB) Native Hawaiian or Other Pacific Islander	0 Participants n=37 Participants	0 Participants n=37 Participants	0 Participants n=74 Participants	0 Participants n=267 Participants	0 Participants n=272 Participants	0 Participants n=6 Participants	0 Participants n=36 Participants	0 Participants n=18 Participants
Race (NIH/OMB) Black or African American	1 Participants n=37 Participants	0 Participants n=37 Participants	1 Participants n=74 Participants	1 Participants n=267 Participants	0 Participants n=272 Participants	0 Participants n=6 Participants	1 Participants n=36 Participants	4 Participants n=18 Participants
Race (NIH/OMB) White	7 Participants n=37 Participants	6 Participants n=37 Participants	9 Participants n=74 Participants	12 Participants n=267 Participants	6 Participants n=272 Participants	30 Participants n=6 Participants	27 Participants n=36 Participants	97 Participants n=18 Participants
Race (NIH/OMB) More than one race	0 Participants n=37 Participants	0 Participants n=37 Participants	0 Participants n=74 Participants	1 Participants n=267 Participants	0 Participants n=272 Participants	0 Participants n=6 Participants	0 Participants n=36 Participants	1 Participants n=18 Participants
Race (NIH/OMB) Unknown or Not Reported	0 Participants n=37 Participants	0 Participants n=37 Participants	0 Participants n=74 Participants	0 Participants n=267 Participants	0 Participants n=272 Participants	1 Participants n=6 Participants	0 Participants n=36 Participants	1 Participants n=18 Participants

PRIMARY outcome

Timeframe: On demand (arm 1): From week 0 up until start of concizumab treatment (week 24); Concizumab (arm 2): From week 0 up until the confirmatory analyses cut-off

Population: FAS included all participants randomised to the new concizumab PPX dosing regimen or on-demand treatment after the treatment pause or allocated to arm 3 or 4 with the new concizumab PPX dosing regimen. This endpoint is only defined for arms 1 and 2 as per protocol.

Outcome measures

Outcome measures
Measure	Arm 1: Previous On Demand (OnD) Treatment: No Prophylaxis (PPX) - Participants With HA n=9 Participants Participants with HA received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HA n=18 Participants Participants with HA received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Extension Phase Arm 1: Concizumab PPX - Participants With HA Participants with HA who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.	Arm 3: Concizumab Non-naive: Concizumab PPX - Participants With HA Participants with HA received a loading dose (participants entering from the phase 2 trial \[N7415-4255\] (NCT03196297) were not to receive a loading dose) of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg.	Arm 4: Concizumab PPX - Participants With HA Participants with HA previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Arm 1: Previous OnD Treatment: No PPX - Participants With HB Participants with HB received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HB Participants with HB received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Arm 4: Concizumab PPX - Participants With HB Participants with HB previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Extension Phase Arm 1: Concizumab PPX - Participants With HB Participants with HB who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.
Haemophilia A Participants Without Inhibitors: Rate of Treated Spontaneous and Traumatic Bleeding Episodes	19.6 Events per year Interval 17.3 to 30.4	2.9 Events per year Interval 0.0 to 5.2	—	—	—	—	—	—	—

PRIMARY outcome

Timeframe: On demand (arm 1): From week 0 up until start of concizumab treatment (week 24); Concizumab (arm 2): From week 0 up until the confirmatory analyses cut-off

Rate of treated spontaneous and traumatic bleeding episodes for haemophilia B participants without inhibitors is presented. The observation period used for reporting this endpoint is OTwoATexBR. It is defined as the time period after the restart where participants are treated by concizumab treatment or are treated by on-demand treatment and additionally have not used factor-containing products not related to treatment of a bleeding episode. The data is reported in the terms of ABR. Week 0 is defined as time of randomisation to on-demand administration after the pause or time of start of the new concizumab dosing regimen. Confirmatory analyses cut-off was defined as when all participants on no PPX (arm 1) had completed the 24-week visit or withdrawn and all participants on concizumab PPX (in arms 2 and 4) had completed the 32-week visit or withdrawn.

Outcome measures

Outcome measures
Measure	Arm 1: Previous On Demand (OnD) Treatment: No Prophylaxis (PPX) - Participants With HA n=12 Participants Participants with HA received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HA n=24 Participants Participants with HA received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Extension Phase Arm 1: Concizumab PPX - Participants With HA Participants with HA who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.	Arm 3: Concizumab Non-naive: Concizumab PPX - Participants With HA Participants with HA received a loading dose (participants entering from the phase 2 trial \[N7415-4255\] (NCT03196297) were not to receive a loading dose) of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg.	Arm 4: Concizumab PPX - Participants With HA Participants with HA previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Arm 1: Previous OnD Treatment: No PPX - Participants With HB Participants with HB received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HB Participants with HB received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Arm 4: Concizumab PPX - Participants With HB Participants with HB previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Extension Phase Arm 1: Concizumab PPX - Participants With HB Participants with HB who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.
Haemophilia B Participants Without Inhibitors: Rate of Treated Spontaneous and Traumatic Bleeding Episodes	14.9 Events per year Interval 3.3 to 22.1	1.6 Events per year Interval 0.0 to 4.8	—	—	—	—	—	—	—

SECONDARY outcome

Timeframe: For previous PPX (study 4322): After an initial period on PPX treatment of at least 24 weeks until end of study (maximum 115 weeks) For concizumab PPX (trial 4307): After an initial 5-8 weeks dose adjustment period and up until 56 week cut off

Population: Intra-participant analysis set (IPAS) included participants in arm 4 that were on a stable PPX regimen for at least 24 weeks in NN7415-4322 and who entered the maintenance period in this trial NN7415-4307. The participants reported in both arms/groups represent the same 29 participants, with results reported from Study 4322 and this study (Study 4307). The endpoint is only defined for arm 4 (previous PPX-study 4322) and arm 4 (concizumab PPX-study 4307) as per protocol.

Rate of treated spontaneous and traumatic bleeding episodes for haemophilia A participants without inhibitors in arm 4 participants who had been on stable PPX at least 24 weeks in study 4322 is presented. The observation period used for reporting this endpoint is on stable treatment without ancillary therapy excluding data before restart (OT stable woATexBR). It is defined as the time period where participants are on stable PPX in study NN7415-4322 combined with the time period after the treatment pause in NN7415-4307 where the same participants are on the maintenance dose and have not used factor-containing products not related to treatment of a bleed. The data is reported in the terms of ABR.

Outcome measures

Outcome measures
Measure	Arm 1: Previous On Demand (OnD) Treatment: No Prophylaxis (PPX) - Participants With HA n=29 Participants Participants with HA received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HA n=29 Participants Participants with HA received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Extension Phase Arm 1: Concizumab PPX - Participants With HA Participants with HA who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.	Arm 3: Concizumab Non-naive: Concizumab PPX - Participants With HA Participants with HA received a loading dose (participants entering from the phase 2 trial \[N7415-4255\] (NCT03196297) were not to receive a loading dose) of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg.	Arm 4: Concizumab PPX - Participants With HA Participants with HA previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Arm 1: Previous OnD Treatment: No PPX - Participants With HB Participants with HB received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HB Participants with HB received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Arm 4: Concizumab PPX - Participants With HB Participants with HB previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Extension Phase Arm 1: Concizumab PPX - Participants With HB Participants with HB who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.
Haemophilia A Participants Without Inhibitors: Rate of Treated Spontaneous and Traumatic Bleeding Episodes	2.2 Events per year Interval 0.8 to 6.2	1.7 Events per year Interval 0.5 to 4.8	—	—	—	—	—	—	—

SECONDARY outcome

Population: IPAS included participants in arm 4 that were on a stable PPX regimen for at least 24 weeks in NN7415-4322 and who entered the maintenance period in this trial NN7415-4307. The participants reported in both arms/groups represent the same 29 participants, with results reported from Study 4322 and this study (Study 4307). The endpoint is only defined for arm 4 (previous PPX-study 4322) and arm 4 (concizumab PPX-study 4307) as per protocol.

Rate of treated spontaneous and traumatic bleeding episodes for haemophilia B participants without inhibitors in arm 4 participants who had been on stable PPX at least 24 weeks in study 4322 is presented. The observation period used for reporting this endpoint is OT stable woATexBR. It is defined as the time period where participants are on stable PPX in study NN7415-4322 combined with the time period after the treatment pause in NN7415-4307 where the same participants are on the maintenance dose and have not used factor-containing products not related to treatment of a bleed. The data is reported in the terms of ABR.

Outcome measures

Outcome measures
Measure	Arm 1: Previous On Demand (OnD) Treatment: No Prophylaxis (PPX) - Participants With HA n=22 Participants Participants with HA received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HA n=22 Participants Participants with HA received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Extension Phase Arm 1: Concizumab PPX - Participants With HA Participants with HA who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.	Arm 3: Concizumab Non-naive: Concizumab PPX - Participants With HA Participants with HA received a loading dose (participants entering from the phase 2 trial \[N7415-4255\] (NCT03196297) were not to receive a loading dose) of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg.	Arm 4: Concizumab PPX - Participants With HA Participants with HA previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Arm 1: Previous OnD Treatment: No PPX - Participants With HB Participants with HB received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HB Participants with HB received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Arm 4: Concizumab PPX - Participants With HB Participants with HB previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Extension Phase Arm 1: Concizumab PPX - Participants With HB Participants with HB who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.
Haemophilia B Participants Without Inhibitors: Rate of Treated Spontaneous and Traumatic Bleeding Episodes	2.1 Events per year Interval 0.9 to 4.2	1.3 Events per year Interval 0.0 to 6.4	—	—	—	—	—	—	—

SECONDARY outcome

Timeframe: On demand (arm 1): From randomisation after the pause (week 0) up until start of concizumab treatment (week 24) Concizumab (arm 2): From start of the new concizumab dosing regimen (week 0) up until the 56 week cut-off

Rate of treated spontaneous bleeding episodes for haemophilia A participants without inhibitors is presented. The observation period used for reporting this endpoint is OTwoATexBR. It is defined as the time period after the restart where participants are treated by concizumab treatment or are treated by on-demand treatment and additionally have not used factor-containing products not related to treatment of a bleeding episode. The data is reported in the terms of ABR. Week 0 is defined as time of randomisation to on-demand administration after the pause or time of start of the new concizumab dosing regimen.

Outcome measures

Outcome measures
Measure	Arm 1: Previous On Demand (OnD) Treatment: No Prophylaxis (PPX) - Participants With HA n=9 Participants Participants with HA received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HA n=18 Participants Participants with HA received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Extension Phase Arm 1: Concizumab PPX - Participants With HA Participants with HA who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.	Arm 3: Concizumab Non-naive: Concizumab PPX - Participants With HA Participants with HA received a loading dose (participants entering from the phase 2 trial \[N7415-4255\] (NCT03196297) were not to receive a loading dose) of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg.	Arm 4: Concizumab PPX - Participants With HA Participants with HA previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Arm 1: Previous OnD Treatment: No PPX - Participants With HB Participants with HB received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HB Participants with HB received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Arm 4: Concizumab PPX - Participants With HB Participants with HB previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Extension Phase Arm 1: Concizumab PPX - Participants With HB Participants with HB who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.
Haemophilia A Participants Without Inhibitors: Rate of Treated Spontaneous Bleeding Episodes	19.3 Events per year Interval 7.2 to 20.5	1.0 Events per year Interval 0.0 to 3.6	—	—	—	—	—	—	—

SECONDARY outcome

Rate of treated spontaneous bleeding episodes for haemophilia B participants without inhibitors is presented. The observation period used for reporting this endpoint is OTwoATexBR. It is defined as the time period after the restart where participants are treated by concizumab treatment or are treated by on-demand treatment and additionally have not used factor-containing products not related to treatment of a bleeding episode. The data is reported in the terms of ABR. Week 0 is defined as time of randomisation to on-demand administration after the pause or time of start of the new concizumab dosing regimen.

Outcome measures

Outcome measures
Measure	Arm 1: Previous On Demand (OnD) Treatment: No Prophylaxis (PPX) - Participants With HA n=12 Participants Participants with HA received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HA n=24 Participants Participants with HA received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Extension Phase Arm 1: Concizumab PPX - Participants With HA Participants with HA who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.	Arm 3: Concizumab Non-naive: Concizumab PPX - Participants With HA Participants with HA received a loading dose (participants entering from the phase 2 trial \[N7415-4255\] (NCT03196297) were not to receive a loading dose) of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg.	Arm 4: Concizumab PPX - Participants With HA Participants with HA previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Arm 1: Previous OnD Treatment: No PPX - Participants With HB Participants with HB received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HB Participants with HB received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Arm 4: Concizumab PPX - Participants With HB Participants with HB previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Extension Phase Arm 1: Concizumab PPX - Participants With HB Participants with HB who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.
Haemophilia B Participants Without Inhibitors: Rate of Treated Spontaneous Bleeding Episodes	10.8 Events per year Interval 3.3 to 17.8	1.0 Events per year Interval 0.0 to 2.5	—	—	—	—	—	—	—

SECONDARY outcome

Rate of treated spontaneous and traumatic joint bleeding episodes for haemophilia A participants without inhibitors is presented. The observation period used for reporting this endpoint is OTwoATexBR. It is defined as the time period after the restart where participants are treated by concizumab treatment or are treated by on-demand treatment and additionally have not used factor-containing products not related to treatment of a bleeding episode. The data is reported in the terms of ABR. Week 0 is defined as time of randomisation to on-demand administration after the pause or time of start of the new concizumab dosing regimen.

Outcome measures

Outcome measures
Measure	Arm 1: Previous On Demand (OnD) Treatment: No Prophylaxis (PPX) - Participants With HA n=9 Participants Participants with HA received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HA n=18 Participants Participants with HA received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Extension Phase Arm 1: Concizumab PPX - Participants With HA Participants with HA who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.	Arm 3: Concizumab Non-naive: Concizumab PPX - Participants With HA Participants with HA received a loading dose (participants entering from the phase 2 trial \[N7415-4255\] (NCT03196297) were not to receive a loading dose) of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg.	Arm 4: Concizumab PPX - Participants With HA Participants with HA previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Arm 1: Previous OnD Treatment: No PPX - Participants With HB Participants with HB received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HB Participants with HB received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Arm 4: Concizumab PPX - Participants With HB Participants with HB previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Extension Phase Arm 1: Concizumab PPX - Participants With HB Participants with HB who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.
Haemophilia A Participants Without Inhibitors: Rate of Treated Spontaneous and Traumatic Joint Bleeding Episodes	13.0 Events per year Interval 10.7 to 28.3	2.0 Events per year Interval 0.0 to 4.0	—	—	—	—	—	—	—

SECONDARY outcome

Rate of treated spontaneous and traumatic joint bleeding episodes for haemophilia B participants without inhibitors is presented. The observation period used for reporting this endpoint is OTwoATexBR. It is defined as the time period after the restart where participants are treated by concizumab treatment or are treated by on-demand treatment and additionally have not used factor-containing products not related to treatment of a bleeding episode. The data is reported in the terms of ABR. Week 0 is defined as time of randomisation to on-demand administration after the pause or time of start of the new concizumab dosing regimen.

Outcome measures

Outcome measures
Measure	Arm 1: Previous On Demand (OnD) Treatment: No Prophylaxis (PPX) - Participants With HA n=12 Participants Participants with HA received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HA n=24 Participants Participants with HA received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Extension Phase Arm 1: Concizumab PPX - Participants With HA Participants with HA who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.	Arm 3: Concizumab Non-naive: Concizumab PPX - Participants With HA Participants with HA received a loading dose (participants entering from the phase 2 trial \[N7415-4255\] (NCT03196297) were not to receive a loading dose) of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg.	Arm 4: Concizumab PPX - Participants With HA Participants with HA previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Arm 1: Previous OnD Treatment: No PPX - Participants With HB Participants with HB received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HB Participants with HB received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Arm 4: Concizumab PPX - Participants With HB Participants with HB previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Extension Phase Arm 1: Concizumab PPX - Participants With HB Participants with HB who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.
Haemophilia B Participants Without Inhibitors: Rate of Treated Spontaneous and Traumatic Joint Bleeding Episodes	10.0 Events per year Interval 3.3 to 18.0	2.0 Events per year Interval 0.0 to 3.7	—	—	—	—	—	—	—

SECONDARY outcome

Rate of treated spontaneous and traumatic target joint bleeding episodes for haemophilia A participants without inhibitors is presented. The observation period used for reporting this endpoint is OTwoATexBR. It is defined as the time period after the restart where participants are treated by concizumab treatment or are treated by on-demand treatment and additionally have not used factor-containing products not related to treatment of a bleeding episode. The data is reported in the terms of ABR. Week 0 is defined as time of randomisation to on-demand administration after the pause or time of start of the new concizumab dosing regimen.

Outcome measures

Outcome measures
Measure	Arm 1: Previous On Demand (OnD) Treatment: No Prophylaxis (PPX) - Participants With HA n=9 Participants Participants with HA received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HA n=18 Participants Participants with HA received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Extension Phase Arm 1: Concizumab PPX - Participants With HA Participants with HA who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.	Arm 3: Concizumab Non-naive: Concizumab PPX - Participants With HA Participants with HA received a loading dose (participants entering from the phase 2 trial \[N7415-4255\] (NCT03196297) were not to receive a loading dose) of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg.	Arm 4: Concizumab PPX - Participants With HA Participants with HA previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Arm 1: Previous OnD Treatment: No PPX - Participants With HB Participants with HB received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HB Participants with HB received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Arm 4: Concizumab PPX - Participants With HB Participants with HB previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Extension Phase Arm 1: Concizumab PPX - Participants With HB Participants with HB who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.
Haemophilia A Participants Without Inhibitors: Rate of Treated Spontaneous and Traumatic Target Joint Bleeding Episodes	4.3 Events per year Interval 1.6 to 13.0	1.7 Events per year Interval 0.0 to 3.0	—	—	—	—	—	—	—

SECONDARY outcome

Rate of treated spontaneous and traumatic target joint bleeding episodes for haemophilia B participants without inhibitors is presented. The observation period used for reporting this endpoint is OTwoATexBR. It is defined as the time period after the restart where participants are treated by concizumab treatment or are treated by on-demand treatment and additionally have not used factor-containing products not related to treatment of a bleeding episode. The data is reported in the terms of ABR. Week 0 is defined as time of randomisation to on-demand administration after the pause or time of start of the new concizumab dosing regimen.

Outcome measures

Outcome measures
Measure	Arm 1: Previous On Demand (OnD) Treatment: No Prophylaxis (PPX) - Participants With HA n=12 Participants Participants with HA received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HA n=24 Participants Participants with HA received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Extension Phase Arm 1: Concizumab PPX - Participants With HA Participants with HA who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.	Arm 3: Concizumab Non-naive: Concizumab PPX - Participants With HA Participants with HA received a loading dose (participants entering from the phase 2 trial \[N7415-4255\] (NCT03196297) were not to receive a loading dose) of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg.	Arm 4: Concizumab PPX - Participants With HA Participants with HA previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Arm 1: Previous OnD Treatment: No PPX - Participants With HB Participants with HB received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HB Participants with HB received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Arm 4: Concizumab PPX - Participants With HB Participants with HB previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Extension Phase Arm 1: Concizumab PPX - Participants With HB Participants with HB who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.
Haemophilia B Participants Without Inhibitors: Rate of Treated Spontaneous and Traumatic Target Joint Bleeding Episodes	2.2 Events per year Interval 0.0 to 8.1	0.7 Events per year Interval 0.0 to 1.5	—	—	—	—	—	—	—

SECONDARY outcome

Timeframe: On demand (arm 1): from week 0 until start of concizumab treatment (week 24). Concizumab (arms 2-4): From week 0 up until the 56 week cut-off. Concizumab (arm 1 extension part): From start of concizumab treatment (week 25) up until the 56 week cut-off

Population: SAS was defined as all participants exposed to concizumab PPX or randomised to on-demand treatment. Overall number of participants analysed = Participants with available data for the outcome measure. The endpoint is applicable for reported arms \[for participants with HA (arm1, arm 2, arm 3, arm 4, arm 1 extension part) and participants with HB (arm 1, arm 2, arm 4, arm 1 extension part)\] only.

Number of thromboembolic events in haemophilia A and haemophilia B participants without inhibitors is presented. The observation period used for reporting this endpoint is on-treatment (OT). It is defined as the time period where participants were considered to be affected by no PPX (on-demand treatment) or concizumab PPX. Week 0 is defined as time of randomisation to on-demand administration after the pause or time of start of the new concizumab dosing regimen.

Outcome measures

Outcome measures
Measure	Arm 1: Previous On Demand (OnD) Treatment: No Prophylaxis (PPX) - Participants With HA n=9 Participants Participants with HA received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HA n=18 Participants Participants with HA received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Extension Phase Arm 1: Concizumab PPX - Participants With HA n=7 Participants Participants with HA who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.	Arm 3: Concizumab Non-naive: Concizumab PPX - Participants With HA n=10 Participants Participants with HA received a loading dose (participants entering from the phase 2 trial \[N7415-4255\] (NCT03196297) were not to receive a loading dose) of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg.	Arm 4: Concizumab PPX - Participants With HA n=52 Participants Participants with HA previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Arm 1: Previous OnD Treatment: No PPX - Participants With HB n=12 Participants Participants with HB received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HB n=24 Participants Participants with HB received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Arm 4: Concizumab PPX - Participants With HB n=30 Participants Participants with HB previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Extension Phase Arm 1: Concizumab PPX - Participants With HB n=10 Participants Participants with HB who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.
Haemophila A and Haemophilia B Participants Without Inhibitors: Number of Thromboembolic Events	0 Events	0 Events	0 Events	0 Events	4 Events	0 Events	0 Events	0 Events	0 Events

SECONDARY outcome

Timeframe: From week 0 to end of trial (up to 384 weeks)

Outcome measures

Outcome data not reported

SECONDARY outcome

Number of hypersensitivity type reactions in haemophilia A and haemophilia B participants without inhibitors is presented. The observation period used for reporting this endpoint is OT. It is defined as the time period where participants were considered to be affected by no PPX (on-demand treatment) or concizumab PPX. Week 0 is defined as time of randomisation to on-demand administration after the pause or time of start of the new concizumab dosing regimen.

Outcome measures

Outcome measures
Measure	Arm 1: Previous On Demand (OnD) Treatment: No Prophylaxis (PPX) - Participants With HA n=9 Participants Participants with HA received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HA n=18 Participants Participants with HA received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Extension Phase Arm 1: Concizumab PPX - Participants With HA n=7 Participants Participants with HA who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.	Arm 3: Concizumab Non-naive: Concizumab PPX - Participants With HA n=10 Participants Participants with HA received a loading dose (participants entering from the phase 2 trial \[N7415-4255\] (NCT03196297) were not to receive a loading dose) of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg.	Arm 4: Concizumab PPX - Participants With HA n=52 Participants Participants with HA previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Arm 1: Previous OnD Treatment: No PPX - Participants With HB n=12 Participants Participants with HB received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HB n=24 Participants Participants with HB received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Arm 4: Concizumab PPX - Participants With HB n=30 Participants Participants with HB previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Extension Phase Arm 1: Concizumab PPX - Participants With HB n=10 Participants Participants with HB who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.
Haemophila A and Haemophilia B Participants Without Inhibitors: Number of Hypersensitivity Type Reactions	0 Reactions	0 Reactions	0 Reactions	0 Reactions	0 Reactions	0 Reactions	0 Reactions	0 Reactions	0 Reactions

SECONDARY outcome

Timeframe: From week 0 to end of trial (up until 384 weeks)

Outcome measures

Outcome data not reported

SECONDARY outcome

Timeframe: On demand (arm 1 main part): from randomisation (week 0) until start of concizumab treatment (week 24). Concizumab (arms 2-4): From week 0 up until the 56 week cut-off. Concizumab (arm 1 extension part): From week 25 up until the 56 week cut-off

Number of injection site reactions in haemophilia A and haemophilia B participants without inhibitors is presented. The observation period used for reporting this endpoint is OT. It is defined as the time period where participants were considered to be affected by no PPX (on-demand treatment) or concizumab PPX. Week 0 is defined as time of randomisation to on-demand administration after the pause or time of start of the new concizumab dosing regimen.

Outcome measures

Outcome measures
Measure	Arm 1: Previous On Demand (OnD) Treatment: No Prophylaxis (PPX) - Participants With HA n=9 Participants Participants with HA received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HA n=18 Participants Participants with HA received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Extension Phase Arm 1: Concizumab PPX - Participants With HA n=7 Participants Participants with HA who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.	Arm 3: Concizumab Non-naive: Concizumab PPX - Participants With HA n=10 Participants Participants with HA received a loading dose (participants entering from the phase 2 trial \[N7415-4255\] (NCT03196297) were not to receive a loading dose) of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg.	Arm 4: Concizumab PPX - Participants With HA n=52 Participants Participants with HA previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Arm 1: Previous OnD Treatment: No PPX - Participants With HB n=12 Participants Participants with HB received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HB n=24 Participants Participants with HB received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Arm 4: Concizumab PPX - Participants With HB n=30 Participants Participants with HB previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Extension Phase Arm 1: Concizumab PPX - Participants With HB n=10 Participants Participants with HB who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.
Haemophila A and Haemophilia B Participants Without Inhibitors: Number of Injection Site Reactions	0 Reactions	1 Reactions	1 Reactions	0 Reactions	14 Reactions	0 Reactions	2 Reactions	28 Reactions	0 Reactions

SECONDARY outcome

Timeframe: From week 0 to end of trial (up until 384 weeks)

Outcome measures

Outcome data not reported

SECONDARY outcome

Timeframe: Concizumab (arms 2-4): From start of concizumab treatment (week 0) up until the 56 week cut-off. Concizumab (arm 1 extension part): From start of concizumab treatment (week 25) up until the 56 week cut-off

Outcome measures

Outcome data not reported

SECONDARY outcome

Timeframe: From week 0 to end of trial (up until 384 weeks)

Outcome measures

Outcome data not reported

SECONDARY outcome

Timeframe: Pre-dose (prior to the concizumab administration at week 24)

Population: SAS was defined as all participants exposed to concizumab PPX or randomised to on-demand treatment. Overall number of participants analysed = Participants with available data for the outcome measure. The endpoint is applicable for reported arms \[for participants with HA (arm 2, arm 3, arm 4) and participants with HB (arm 2, arm 4)\] only.

Ctrough for haemophilia A and haemophilia B participants without inhibitors is presented. The observation period used for reporting this endpoint is on-treatment without data before re-start (OTexBR). It is defined as the time period after restart where participants were considered to be affected by no PPX (on-demand treatment) or treatment with the new concizumab regimen.

Outcome measures

Outcome measures
Measure	Arm 1: Previous On Demand (OnD) Treatment: No Prophylaxis (PPX) - Participants With HA n=15 Participants Participants with HA received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HA n=7 Participants Participants with HA received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Extension Phase Arm 1: Concizumab PPX - Participants With HA n=26 Participants Participants with HA who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.	Arm 3: Concizumab Non-naive: Concizumab PPX - Participants With HA n=45 Participants Participants with HA received a loading dose (participants entering from the phase 2 trial \[N7415-4255\] (NCT03196297) were not to receive a loading dose) of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg.	Arm 4: Concizumab PPX - Participants With HA n=23 Participants Participants with HA previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Arm 1: Previous OnD Treatment: No PPX - Participants With HB Participants with HB received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HB Participants with HB received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Arm 4: Concizumab PPX - Participants With HB Participants with HB previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Extension Phase Arm 1: Concizumab PPX - Participants With HB Participants with HB who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.
Haemophila A and Haemophilia B Participants Without Inhibitors: Pre-dose (Trough) Concizumab Plasma Concentration (Ctrough)	767.7 nanograms per milliliter (ng/mL) Geometric Coefficient of Variation 112.4	612.3 nanograms per milliliter (ng/mL) Geometric Coefficient of Variation 274.9	719.7 nanograms per milliliter (ng/mL) Geometric Coefficient of Variation 189.7	729.3 nanograms per milliliter (ng/mL) Geometric Coefficient of Variation 158.6	413.6 nanograms per milliliter (ng/mL) Geometric Coefficient of Variation 234.7	—	—	—	—

SECONDARY outcome

Timeframe: Pre-dose (prior to the concizumab administration at week 24)

Population: SAS was defined as all participants exposed to concizumab PPX or randomised to on-demand treatment. Overall number of participants analysed = Participants with available data for the outcome measure. The endpoint is applicable for reported arms \[for participants with HA (arm 1, arm 2, arm 3, arm 4) and participants with HB (arm 1, arm 2, arm 4)\] only.

Pre-dose thrombin peak for haemophilia A and haemophilia B participants without inhibitors is presented. The observation period used for reporting this endpoint is OTexBR. It is defined as the time period after restart where participants were considered to be affected by no PPX (on-demand treatment) or treatment with the new concizumab regimen.

Outcome measures

Outcome measures
Measure	Arm 1: Previous On Demand (OnD) Treatment: No Prophylaxis (PPX) - Participants With HA n=7 Participants Participants with HA received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HA n=15 Participants Participants with HA received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Extension Phase Arm 1: Concizumab PPX - Participants With HA n=9 Participants Participants with HA who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.	Arm 3: Concizumab Non-naive: Concizumab PPX - Participants With HA n=7 Participants Participants with HA received a loading dose (participants entering from the phase 2 trial \[N7415-4255\] (NCT03196297) were not to receive a loading dose) of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg.	Arm 4: Concizumab PPX - Participants With HA n=43 Participants Participants with HA previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Arm 1: Previous OnD Treatment: No PPX - Participants With HB n=22 Participants Participants with HB received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HB n=26 Participants Participants with HB received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Arm 4: Concizumab PPX - Participants With HB Participants with HB previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Extension Phase Arm 1: Concizumab PPX - Participants With HB Participants with HB who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.
Haemophila A and Haemophilia B Participants Without Inhibitors: Pre-dose Thrombin Peak	19.9 nanomoles per liter (nmol/L) Geometric Coefficient of Variation 117.7	98.3 nanomoles per liter (nmol/L) Geometric Coefficient of Variation 53.3	9.6 nanomoles per liter (nmol/L) Geometric Coefficient of Variation 110.7	92.3 nanomoles per liter (nmol/L) Geometric Coefficient of Variation 69.0	92.1 nanomoles per liter (nmol/L) Geometric Coefficient of Variation 57.8	54.6 nanomoles per liter (nmol/L) Geometric Coefficient of Variation 111.9	79.4 nanomoles per liter (nmol/L) Geometric Coefficient of Variation 57.1	—	—

SECONDARY outcome

Timeframe: Pre-dose (prior to the concizumab administration at week 24)

Population: SAS was defined as all participants exposed to concizumab PPX or randomised to on-demand treatment. Overall number of participants analysed = Participants with available data for the outcome measure. The endpoint is applicable for reported arms \[for participants with HA (arm 1, arm 2, arm 3, arm 4) and participants with HB (arm 1, arm 2, arm 4)\] only.

Pre-dose free TFPI for haemophilia A and haemophilia B participants without inhibitors is presented. The observation period used for reporting this endpoint is OTexBR. It is defined as the time period after restart where participants were considered to be affected by no PPX (on-demand treatment) or treatment with the new concizumab regimen.

Outcome measures

Outcome measures
Measure	Arm 1: Previous On Demand (OnD) Treatment: No Prophylaxis (PPX) - Participants With HA n=7 Participants Participants with HA received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HA n=16 Participants Participants with HA received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Extension Phase Arm 1: Concizumab PPX - Participants With HA n=11 Participants Participants with HA who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.	Arm 3: Concizumab Non-naive: Concizumab PPX - Participants With HA n=7 Participants Participants with HA received a loading dose (participants entering from the phase 2 trial \[N7415-4255\] (NCT03196297) were not to receive a loading dose) of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg.	Arm 4: Concizumab PPX - Participants With HA n=44 Participants Participants with HA previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Arm 1: Previous OnD Treatment: No PPX - Participants With HB n=23 Participants Participants with HB received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HB n=26 Participants Participants with HB received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Arm 4: Concizumab PPX - Participants With HB Participants with HB previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Extension Phase Arm 1: Concizumab PPX - Participants With HB Participants with HB who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.
Haemophila A and Haemophilia B Participants Without Inhibitors: Pre-dose Free Tissue Factor Pathway Inhibitor (TFPI) Concentration	70.1 ng/mL Geometric Coefficient of Variation 12.3	11.1 ng/mL Geometric Coefficient of Variation 74.8	88.1 ng/mL Geometric Coefficient of Variation 14.6	15.9 ng/mL Geometric Coefficient of Variation 75.8	9.6 ng/mL Geometric Coefficient of Variation 87.9	16.3 ng/mL Geometric Coefficient of Variation 130.3	10.5 ng/mL Geometric Coefficient of Variation 107.4	—	—

SECONDARY outcome

Timeframe: Pre-dose, Week 24: 3 hours (h), 6h, 9h, 24h

Population: SAS was defined as all participants exposed to concizumab PPX or randomised to on-demand treatment. Overall number of participants analysed = Participants with available data for the outcome measure. The endpoint is applicable for reported arms \[for participants with HA (arm 2, arm 3, arm 4) and participants with HB (arm 2, arm 4)\] only.

Cmax for haemophilia A and haemophilia B participants without inhibitors is presented. The observation period used for reporting this endpoint is OTexBR. It is defined as the time period after restart where participants were considered to be affected by no PPX (on-demand treatment) or treatment with the new concizumab regimen.

Outcome measures

Outcome measures
Measure	Arm 1: Previous On Demand (OnD) Treatment: No Prophylaxis (PPX) - Participants With HA n=10 Participants Participants with HA received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HA n=5 Participants Participants with HA received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Extension Phase Arm 1: Concizumab PPX - Participants With HA n=22 Participants Participants with HA who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.	Arm 3: Concizumab Non-naive: Concizumab PPX - Participants With HA n=36 Participants Participants with HA received a loading dose (participants entering from the phase 2 trial \[N7415-4255\] (NCT03196297) were not to receive a loading dose) of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg.	Arm 4: Concizumab PPX - Participants With HA n=11 Participants Participants with HA previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Arm 1: Previous OnD Treatment: No PPX - Participants With HB Participants with HB received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HB Participants with HB received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Arm 4: Concizumab PPX - Participants With HB Participants with HB previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Extension Phase Arm 1: Concizumab PPX - Participants With HB Participants with HB who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.
Haemophila A and Haemophilia B Participants Without Inhibitors: Maximum Concizumab Plasma Concentration (Cmax)	1158.2 ng/mL Geometric Coefficient of Variation 61.9	583.4 ng/mL Geometric Coefficient of Variation 282.2	828.0 ng/mL Geometric Coefficient of Variation 111.2	1029.7 ng/mL Geometric Coefficient of Variation 130.9	689.2 ng/mL Geometric Coefficient of Variation 190.5	—	—	—	—

SECONDARY outcome

Timeframe: Pre-dose, Week 24: 3 hours (h), 6h, 9h, 24h

Population: SAS was defined as all participants exposed to concizumab PPX or randomised to on-demand treatment. Overall number of participants analysed = Participants with available data for the outcome measure. The endpoint is applicable for reported arms \[for participants with HA (arm 2, arm 3, arm 4) and participants with HB (arm 2, arm 4)\] only.

AUC for haemophilia A and haemophilia B participants without inhibitors is presented. The observation period used for reporting this endpoint is OTexBR. It is defined as the time period after restart where participants were considered to be affected by no PPX (on-demand treatment) or treatment with the new concizumab regimen.

Outcome measures

Outcome measures
Measure	Arm 1: Previous On Demand (OnD) Treatment: No Prophylaxis (PPX) - Participants With HA n=10 Participants Participants with HA received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HA n=5 Participants Participants with HA received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Extension Phase Arm 1: Concizumab PPX - Participants With HA n=22 Participants Participants with HA who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.	Arm 3: Concizumab Non-naive: Concizumab PPX - Participants With HA n=36 Participants Participants with HA received a loading dose (participants entering from the phase 2 trial \[N7415-4255\] (NCT03196297) were not to receive a loading dose) of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg.	Arm 4: Concizumab PPX - Participants With HA n=11 Participants Participants with HA previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Arm 1: Previous OnD Treatment: No PPX - Participants With HB Participants with HB received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HB Participants with HB received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Arm 4: Concizumab PPX - Participants With HB Participants with HB previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Extension Phase Arm 1: Concizumab PPX - Participants With HB Participants with HB who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.
Haemophila A and Haemophilia B Participants Without Inhibitors: Area Under the Concizumab Plasma Concentration-time Curve (AUC)	23582.5 nanogramshour/milliliter (nghr/mL) Geometric Coefficient of Variation 68.2	11082.7 nanogramshour/milliliter (nghr/mL) Geometric Coefficient of Variation 268.0	16191.8 nanogramshour/milliliter (nghr/mL) Geometric Coefficient of Variation 118.2	20355.6 nanogramshour/milliliter (nghr/mL) Geometric Coefficient of Variation 135.2	13020.5 nanogramshour/milliliter (nghr/mL) Geometric Coefficient of Variation 182.7	—	—	—	—

Adverse Events

Arm 1: Previous On Demand (OnD) Treatment: No Prophylaxis (PPX) - Participants With HA

Serious events: 0 serious events

Other events: 2 other events

Deaths: 0 deaths

Extension Phase Arm 1: Concizumab PPX - Participants With HA

Serious events: 0 serious events

Other events: 5 other events

Deaths: 0 deaths

Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HA

Serious events: 1 serious events

Other events: 10 other events

Deaths: 0 deaths

Arm 3: Concizumab Non-naive: Concizumab PPX - Participants With HA

Serious events: 1 serious events

Other events: 8 other events

Deaths: 0 deaths

Arm 4: Concizumab PPX - Participants With HA

Serious events: 10 serious events

Other events: 40 other events

Deaths: 1 deaths

Arm 1: Previous OnD Treatment: No PPX - Participants With HB

Serious events: 2 serious events

Other events: 2 other events

Deaths: 0 deaths

Extension Phase Arm 1: Concizumab PPX - Participants With HB

Serious events: 0 serious events

Other events: 5 other events

Deaths: 0 deaths

Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HB

Serious events: 6 serious events

Other events: 12 other events

Deaths: 0 deaths

Arm 4: Concizumab PPX - Participants With HB

Serious events: 2 serious events

Other events: 24 other events

Deaths: 0 deaths

Serious adverse events

Serious adverse events
Measure	Arm 1: Previous On Demand (OnD) Treatment: No Prophylaxis (PPX) - Participants With HA n=9 participants at risk Participants with HA received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Extension Phase Arm 1: Concizumab PPX - Participants With HA n=7 participants at risk Participants with HA who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HA n=18 participants at risk Participants with HA received a loading dose of 1.0 milligrams per kilograms (mg/kg) concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Arm 3: Concizumab Non-naive: Concizumab PPX - Participants With HA n=10 participants at risk Participants with HA received a loading dose (participants entering from the phase 2 trial \[N7415-4255\] (NCT03196297) were not to receive a loading dose) of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg.	Arm 4: Concizumab PPX - Participants With HA n=52 participants at risk Participants with HA previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Arm 1: Previous OnD Treatment: No PPX - Participants With HB n=12 participants at risk Participants with HB received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Extension Phase Arm 1: Concizumab PPX - Participants With HB n=10 participants at risk Participants with HB who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HB n=24 participants at risk Participants with HB received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Arm 4: Concizumab PPX - Participants With HB n=30 participants at risk Participants with HB previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.
Injury, poisoning and procedural complications Abdominal injury	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	4.2% 1/24 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Cardiac disorders Acute myocardial infarction	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	1.9% 1/52 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Musculoskeletal and connective tissue disorders Arthropathy	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	8.3% 1/12 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Gastrointestinal disorders Ascites	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	8.3% 1/12 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Infections and infestations COVID-19	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	1.9% 1/52 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	8.3% 1/12 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Cardiac disorders Cardiac failure	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 1/10 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Injury, poisoning and procedural complications Craniocerebral injury	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	1.9% 1/52 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Vascular disorders Deep vein thrombosis	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	1.9% 1/52 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Nervous system disorders Dizziness	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	4.2% 1/24 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Gastrointestinal disorders Enterocolitis	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	4.2% 1/24 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Musculoskeletal and connective tissue disorders Haematoma muscle	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	1.9% 1/52 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Musculoskeletal and connective tissue disorders Haemophilic arthropathy	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	4.2% 1/24 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Nervous system disorders Hemiparesis	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	1.9% 1/52 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Hepatobiliary disorders Hepatic failure	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	8.3% 1/12 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Hepatobiliary disorders Hepatorenal syndrome	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	8.3% 1/12 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Gastrointestinal disorders Intra-abdominal haemorrhage	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	1.9% 1/52 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	3.3% 1/30 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Hepatobiliary disorders Liver disorder	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	8.3% 1/12 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Infections and infestations Lower respiratory tract infection	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	4.2% 1/24 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Infections and infestations Pneumonia	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	1.9% 1/52 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	4.2% 1/24 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Injury, poisoning and procedural complications Post procedural haematoma	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	4.2% 1/24 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Respiratory, thoracic and mediastinal disorders Pulmonary embolism	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	1.9% 1/52 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Renal and urinary disorders Renal failure	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	8.3% 1/12 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Gastrointestinal disorders Retroperitoneal haemorrhage	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	1.9% 1/52 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Psychiatric disorders Suicidal ideation	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	3.3% 1/30 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Vascular disorders Superficial vein thrombosis	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	1.9% 1/52 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Renal and urinary disorders Urinary retention	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	4.2% 1/24 • Number of events 2 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Gastrointestinal disorders Abdominal pain	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	5.6% 1/18 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Injury, poisoning and procedural complications Cartilage injury	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	1.9% 1/52 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Infections and infestations Gastroenteritis rotavirus	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	1.9% 1/52 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Injury, poisoning and procedural complications Ligament sprain	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	1.9% 1/52 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Renal and urinary disorders Renal haematoma	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	4.2% 1/24 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Injury, poisoning and procedural complications Traumatic haematoma	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	1.9% 1/52 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Renal and urinary disorders Ureterolithiasis	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	1.9% 1/52 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.

Other adverse events

Other adverse events
Measure	Arm 1: Previous On Demand (OnD) Treatment: No Prophylaxis (PPX) - Participants With HA n=9 participants at risk Participants with HA received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Extension Phase Arm 1: Concizumab PPX - Participants With HA n=7 participants at risk Participants with HA who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HA n=18 participants at risk Participants with HA received a loading dose of 1.0 milligrams per kilograms (mg/kg) concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Arm 3: Concizumab Non-naive: Concizumab PPX - Participants With HA n=10 participants at risk Participants with HA received a loading dose (participants entering from the phase 2 trial \[N7415-4255\] (NCT03196297) were not to receive a loading dose) of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg.	Arm 4: Concizumab PPX - Participants With HA n=52 participants at risk Participants with HA previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.	Arm 1: Previous OnD Treatment: No PPX - Participants With HB n=12 participants at risk Participants with HB received their on-demand treatment for a minimum of 24 weeks. After treatment pause, participants initially randomised to arm 1 were to enter arm 4. Subsequently, following the main phase, participants transitioned to the new dosing regimen for concizumab and were scheduled to undergo concizumab prophylaxis in the trial's extension phase.	Extension Phase Arm 1: Concizumab PPX - Participants With HB n=10 participants at risk Participants with HB who completed the main part were offered concizumab PPX in the extension part for up to an additional 353 weeks.	Arm 2: Previous OnD Treatment: Concizumab PPX - Participants With HB n=24 participants at risk Participants with HB received a loading dose of 1.0 mg/kg concizumab subcutaneously followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could be increased or decreased in dose to 0.25 mg/kg or 0.15 mg/kg concizumab or stayed at 0.20mg/kg. After treatment pause, participants initially randomised to arm 2 were to enter arm 4.	Arm 4: Concizumab PPX - Participants With HB n=30 participants at risk Participants with HB previously on PPX with factor products and a minimum of 24 weeks of observation in the non interventional study 4322 (NCT03741881) were allocated to Arm 4. Additionally, Arm 4 also included participants who were randomised to arms 1 (no PPX) and 2 (concizumab PPX) before the treatment pause, participants who were in the phase 2 trial 4255 at the time of the treatment pause, and who had subsequently completed the trial when concizumab treatment was restarted and on-demand participants included after arms 1 and 2 were closed. The original dosing regimen was a loading s.c. dose of 1.0 mg/kg concizumab on the first day of treatment, followed by a maintenance dose of 0.25 mg/kg concizumab given as a daily s.c. injection from the second day and onwards. After the concizumab treatment pause and the treatment restart, participants received a loading dose of 1.0 mg/kg concizumab at visit 2a followed by an initial daily dose of 0.20 mg/kg concizumab from treatment day 2. Within an initial 5-8-week maintenance dose setting period on 0.20 mg/kg concizumab, the participants could have their dose increased to 0.25 mg/kg or decreased to 0.15 mg/kg concizumab or they could remain at a dose of 0.20 mg/kg.
Gastrointestinal disorders Abdominal pain upper	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	5.8% 3/52 • Number of events 3 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	3.3% 1/30 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Skin and subcutaneous tissue disorders Acne	11.1% 1/9 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	1.9% 1/52 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Respiratory, thoracic and mediastinal disorders Aphonia	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 1/10 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Musculoskeletal and connective tissue disorders Arthralgia	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	20.0% 2/10 • Number of events 2 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	9.6% 5/52 • Number of events 5 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 1/10 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	12.5% 3/24 • Number of events 5 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Musculoskeletal and connective tissue disorders Arthropathy	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	8.3% 1/12 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Cardiac disorders Atrial fibrillation	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 1/10 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Musculoskeletal and connective tissue disorders Back pain	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 1/10 • Number of events 4 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	1.9% 1/52 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 1/10 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	3.3% 1/30 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Investigations Bacterial test positive	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	8.3% 1/12 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Infections and infestations COVID-19	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	11.1% 2/18 • Number of events 2 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	30.0% 3/10 • Number of events 3 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	26.9% 14/52 • Number of events 14 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	12.5% 3/24 • Number of events 3 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	30.0% 9/30 • Number of events 9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Nervous system disorders Cognitive disorder	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 1/10 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Gastrointestinal disorders Dental caries	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	8.3% 2/24 • Number of events 2 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	3.3% 1/30 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Skin and subcutaneous tissue disorders Dermatitis contact	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 1/10 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	1.9% 1/52 • Number of events 2 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Product Issues Device physical property issue	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 1/10 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Nervous system disorders Dizziness	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 1/10 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Skin and subcutaneous tissue disorders Dry skin	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 1/10 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Injury, poisoning and procedural complications Fall	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 1/10 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	1.9% 1/52 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	4.2% 1/24 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	3.3% 1/30 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Investigations Fibrin D dimer increased	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 1/10 • Number of events 2 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	15.4% 8/52 • Number of events 8 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	4.2% 1/24 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 3/30 • Number of events 3 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Investigations Fibrinolysis	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	5.6% 1/18 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	1.9% 1/52 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	4.2% 1/24 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Musculoskeletal and connective tissue disorders Flank pain	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	5.6% 1/18 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Gastrointestinal disorders Gastritis	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	5.6% 1/18 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Nervous system disorders Headache	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	20.0% 2/10 • Number of events 4 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	3.8% 2/52 • Number of events 3 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	12.5% 3/24 • Number of events 3 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	6.7% 2/30 • Number of events 6 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Investigations Hepatic enzyme increased	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 1/10 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	4.2% 1/24 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Vascular disorders Hypertension	11.1% 1/9 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 1/10 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	5.8% 3/52 • Number of events 3 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 1/10 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	4.2% 1/24 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	6.7% 2/30 • Number of events 2 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Nervous system disorders Hypoaesthesia	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	14.3% 1/7 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	5.6% 1/18 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Gastrointestinal disorders Inflammatory bowel disease	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	5.6% 1/18 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Infections and infestations Influenza	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	14.3% 1/7 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	5.6% 1/18 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	3.8% 2/52 • Number of events 2 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	4.2% 1/24 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	13.3% 4/30 • Number of events 6 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
General disorders Injection site erythema	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	5.8% 3/52 • Number of events 4 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 3/30 • Number of events 10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
General disorders Injection site haematoma	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	1.9% 1/52 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	6.7% 2/30 • Number of events 2 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
General disorders Injection site rash	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	5.6% 1/18 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	3.3% 1/30 • Number of events 5 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
General disorders Injection site reaction	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	3.8% 2/52 • Number of events 3 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	4.2% 1/24 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 3/30 • Number of events 5 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Eye disorders Keratitis	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	5.6% 1/18 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Cardiac disorders Left ventricular dysfunction	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	8.3% 1/12 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Musculoskeletal and connective tissue disorders Musculoskeletal discomfort	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 1/10 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Musculoskeletal and connective tissue disorders Myalgia	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	1.9% 1/52 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 3/30 • Number of events 5 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Cardiac disorders Myocardial ischaemia	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	8.3% 1/12 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Respiratory, thoracic and mediastinal disorders Nasal congestion	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 1/10 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 1/10 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	4.2% 1/24 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	3.3% 1/30 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Infections and infestations Nasopharyngitis	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	5.6% 1/18 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 1/10 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	15.4% 8/52 • Number of events 9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 1/10 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	8.3% 2/24 • Number of events 2 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Musculoskeletal and connective tissue disorders Neck pain	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 1/10 • Number of events 4 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	3.3% 1/30 • Number of events 2 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Blood and lymphatic system disorders Neutropenia	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	5.6% 1/18 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	1.9% 1/52 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
General disorders Oedema peripheral	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	3.8% 2/52 • Number of events 3 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	8.3% 1/12 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 1/10 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	3.3% 1/30 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Reproductive system and breast disorders Prostatitis	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 1/10 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Investigations Prothrombin fragment 1.2 increased	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	20.0% 2/10 • Number of events 4 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	11.5% 6/52 • Number of events 6 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	6.7% 2/30 • Number of events 2 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Infections and infestations Pyelonephritis	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 1/10 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Infections and infestations Pyoderma	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 1/10 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
General disorders Pyrexia	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	20.0% 2/10 • Number of events 2 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	5.8% 3/52 • Number of events 5 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	6.7% 2/30 • Number of events 4 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Infections and infestations Rhinitis	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	14.3% 1/7 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	3.8% 2/52 • Number of events 2 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	3.3% 1/30 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Injury, poisoning and procedural complications Skin laceration	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	1.9% 1/52 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	6.7% 2/30 • Number of events 2 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Injury, poisoning and procedural complications Subcutaneous haematoma	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 1/10 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Ear and labyrinth disorders Tinnitus	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 1/10 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	1.9% 1/52 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Gastrointestinal disorders Toothache	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	3.8% 2/52 • Number of events 2 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	4.2% 1/24 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 3/30 • Number of events 4 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Injury, poisoning and procedural complications Upper limb fracture	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 1/10 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Infections and infestations Upper respiratory tract infection	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	14.3% 1/7 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	11.1% 2/18 • Number of events 2 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	9.6% 5/52 • Number of events 7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	8.3% 2/24 • Number of events 3 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 3/30 • Number of events 4 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Infections and infestations Urinary tract infection	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	8.3% 1/12 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	3.3% 1/30 • Number of events 2 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Immune system disorders Allergy to arthropod bite	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	14.3% 1/7 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	1.9% 1/52 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	6.7% 2/30 • Number of events 2 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Gastrointestinal disorders Diarrhoea	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	5.6% 1/18 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	1.9% 1/52 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	4.2% 1/24 • Number of events 2 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	3.3% 1/30 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Ear and labyrinth disorders Excessive cerumen production	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	14.3% 1/7 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Hepatobiliary disorders Hepatic steatosis	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	5.8% 3/52 • Number of events 3 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Injury, poisoning and procedural complications Humerus fracture	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	14.3% 1/7 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
General disorders Injection site mass	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	14.3% 1/7 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
General disorders Injection site pain	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	3.8% 2/52 • Number of events 2 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	6.7% 2/30 • Number of events 2 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Infections and infestations Laryngitis	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	1.9% 1/52 • Number of events 2 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	6.7% 2/30 • Number of events 2 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Respiratory, thoracic and mediastinal disorders Nasal septum deviation	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	14.3% 1/7 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Skin and subcutaneous tissue disorders Pruritus allergic	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	5.6% 1/18 • Number of events 2 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Infections and infestations Respiratory tract infection	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	5.8% 3/52 • Number of events 3 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Respiratory, thoracic and mediastinal disorders Rhinitis allergic	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	14.3% 1/7 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Skin and subcutaneous tissue disorders Rosacea	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	14.3% 1/7 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Investigations SARS-CoV-2 test positive	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	3.8% 2/52 • Number of events 2 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 3/30 • Number of events 3 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.
Injury, poisoning and procedural complications Sunburn	0.00% 0/9 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/7 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/18 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	10.0% 1/10 • Number of events 1 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/52 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/12 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/10 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/24 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.	0.00% 0/30 • Up to week 56 cut-off Safety analysis set (SAS) included all participants exposed to concizumab prophylaxis (PPX) or randomised to on-demand treatment. All presented AEs were treatment emergent AEs (TEAEs). TEAEs were defined as AEs which initiated or worsened during the time period where participants were considered to be affected by on demand treatment or concizumab treatment.

Additional Information

Clinical Reporting Office (2834)

Novo Nordisk A/S

Phone: (+1) 866-867-7178

Email: [email protected]

Results disclosure agreements

Principal investigator is a sponsor employee At the end of the trial, one or more scientific publications may be prepared collaboratively by the investigator(s) and Novo Nordisk. Novo Nordisk reserves the right to postpone publication and/or communication for up to 60 days to protect intellectual property.
Publication restrictions are in place

Restriction type: OTHER