Trial Outcomes & Findings for Phase III Trial With Caplacizumab in Patients With Acquired Thrombotic Thrombocytopenic Purpura (NCT NCT02553317)

NCT ID: NCT02553317

Last Updated: 2023-04-05

Results Overview

Platelet count response was defined as initial platelet count ≥ 150,000/μL with subsequent stop of daily PE within 5 days. It refers to the first time both conditions, platelet count ≥ 150,000/μL and the stop of daily PE within 5 days, were met.

• Recruitment status: COMPLETED
• Study phase: PHASE3
• Target enrollment: 145 participants
• Primary outcome timeframe: Only data from the DB daily PE period (median = 5 days) up to the cut-off were used. The cut-off point was defined by whichever occured first: 1) 45 days of daily PE after start of study drug, 2) stop of daily PE, 3) stop of study drug (median = 34 days)
• Results posted on: 2023-04-05

Participant Flow

A total of 145 subjects was randomized at 55 sites located in Europe (34 sites; 91 subjects), Asia (4 sites, 6 subjects), Australia (3 sites, 3 subjects), and North America (14 sites; 45 subjects). Consent was obtained from the first subject on 19 November 2015; the last subject completed the final visit on 16 August 2017.

Of the 149 subjects screened, 4 were screen failures and 145 were randomly assigned to treatment (Intent-to-treat [ITT] population). All, except 1 subject who withdrew consent, received study drug and were included in the safety population and in the modified ITT (mITT) population.

Participant milestones

Measure	Caplacizumab Caplacizumab 10 mg once daily Caplacizumab: * First day of treatment: 10 mg intravenous (i.v.) injection prior to plasma exchange (PE) followed by a 10 mg subcutaneous (s.c.) injection (in the abdominal region) after completion of PE on that day. * Subsequent days of treatment during PE: daily 10 mg s.c. injection (in the abdominal region) following PE. * Treatment after PE period: daily 10 mg s.c. injections for 30 days. If the underlying immunological disease was not resolved, treatment could be extended for a maximum of 4 additional 1-week periods (i.e., 28 days) and was to be accompanied by optimization of immunosuppression.	Placebo Placebo once daily Placebo: * First day of treatment: i.v. injection prior to PE followed by a s.c. injection (in the abdominal region) after completion of PE on that day. * Subsequent days of treatment during PE: daily s.c. injection (in the abdominal region) following PE. * Treatment after PE period: daily s.c. injections for 30 days. If the underlying immunological disease was not resolved, treatment could be extended for a maximum of 4 additional 1-week periods (i.e., 28 days) and was to be accompanied by optimization of immunosuppression.
Overall Treatment Period + Follow-up STARTED	72	73
Overall Treatment Period + Follow-up Received Study Drug	71	73
Overall Treatment Period + Follow-up COMPLETED	58	50
Overall Treatment Period + Follow-up NOT COMPLETED	14	23
Double-blind (DB) Treatment Period STARTED	71	73
Double-blind (DB) Treatment Period COMPLETED	68	65
Double-blind (DB) Treatment Period NOT COMPLETED	3	8
Open-label (OL) Treatment Period STARTED	2	26
Open-label (OL) Treatment Period COMPLETED	2	22
Open-label (OL) Treatment Period NOT COMPLETED	0	4

Reasons for withdrawal

Measure	Caplacizumab Caplacizumab 10 mg once daily Caplacizumab: * First day of treatment: 10 mg intravenous (i.v.) injection prior to plasma exchange (PE) followed by a 10 mg subcutaneous (s.c.) injection (in the abdominal region) after completion of PE on that day. * Subsequent days of treatment during PE: daily 10 mg s.c. injection (in the abdominal region) following PE. * Treatment after PE period: daily 10 mg s.c. injections for 30 days. If the underlying immunological disease was not resolved, treatment could be extended for a maximum of 4 additional 1-week periods (i.e., 28 days) and was to be accompanied by optimization of immunosuppression.	Placebo Placebo once daily Placebo: * First day of treatment: i.v. injection prior to PE followed by a s.c. injection (in the abdominal region) after completion of PE on that day. * Subsequent days of treatment during PE: daily s.c. injection (in the abdominal region) following PE. * Treatment after PE period: daily s.c. injections for 30 days. If the underlying immunological disease was not resolved, treatment could be extended for a maximum of 4 additional 1-week periods (i.e., 28 days) and was to be accompanied by optimization of immunosuppression.
Overall Treatment Period + Follow-up Withdrawn by legal representative	0	1
Overall Treatment Period + Follow-up Physician Decision	2	4
Overall Treatment Period + Follow-up Non-compliance with study drug	0	1
Overall Treatment Period + Follow-up Death	1	3
Overall Treatment Period + Follow-up Adverse Event	6	5
Overall Treatment Period + Follow-up Withdrawal by Subject	4	5
Overall Treatment Period + Follow-up Lost to Follow-up	0	1
Overall Treatment Period + Follow-up Diagnosed as non-TTP patient	1	1
Overall Treatment Period + Follow-up Protocol Violation	0	1
Overall Treatment Period + Follow-up Withdrew treatment, agreed for ET visit	0	1

Baseline Characteristics

Phase III Trial With Caplacizumab in Patients With Acquired Thrombotic Thrombocytopenic Purpura

Baseline characteristics by cohort

Measure	Caplacizumab n=72 Participants Caplacizumab 10 mg once daily Caplacizumab: * First day of treatment: 10 mg intravenous (i.v.) injection prior to PE followed by a 10 mg subcutaneous (s.c.) injection (in the abdominal region) after completion of PE on that day. * Subsequent days of treatment during PE: daily 10 mg s.c. injection (in the abdominal region) following PE. * Treatment after PE period: daily 10 mg s.c. injections for 30 days. If the underlying immunological disease was not resolved, treatment could be extended for a maximum of 4 additional 1-week periods (i.e., 28 days) and was to be accompanied by optimization of immunosuppression.	Placebo n=73 Participants Placebo once daily Placebo: * First day of treatment: i.v. injection prior to PE followed by a s.c. injection (in the abdominal region) after completion of PE on that day. * Subsequent days of treatment during PE: daily s.c. injection (in the abdominal region) following PE. * Treatment after PE period: daily s.c. injections for 30 days. If the underlying immunological disease was not resolved, treatment could be extended for a maximum of 4 additional 1-week periods (i.e., 28 days) and was to be accompanied by optimization of immunosuppression.	Total n=145 Participants Total of all reporting groups
Age, Categorical <=18 years	0 Participants n=5 Participants	0 Participants n=7 Participants	0 Participants n=5 Participants
Age, Categorical Between 18 and 65 years	68 Participants n=5 Participants	65 Participants n=7 Participants	133 Participants n=5 Participants
Age, Categorical >=65 years	4 Participants n=5 Participants	8 Participants n=7 Participants	12 Participants n=5 Participants
Age, Continuous	44.9 years STANDARD_DEVIATION 13.46 • n=5 Participants	47.3 years STANDARD_DEVIATION 14.07 • n=7 Participants	46.1 years STANDARD_DEVIATION 13.78 • n=5 Participants
Sex: Female, Male Female	49 Participants n=5 Participants	51 Participants n=7 Participants	100 Participants n=5 Participants
Sex: Female, Male Male	23 Participants n=5 Participants	22 Participants n=7 Participants	45 Participants n=5 Participants
Ethnicity (NIH/OMB) Hispanic or Latino	4 Participants n=5 Participants	2 Participants n=7 Participants	6 Participants n=5 Participants
Ethnicity (NIH/OMB) Not Hispanic or Latino	65 Participants n=5 Participants	62 Participants n=7 Participants	127 Participants n=5 Participants
Ethnicity (NIH/OMB) Unknown or Not Reported	3 Participants n=5 Participants	9 Participants n=7 Participants	12 Participants n=5 Participants
Region of Enrollment Hungary	4 Participants n=5 Participants	3 Participants n=7 Participants	7 Participants n=5 Participants
Region of Enrollment United States	10 Participants n=5 Participants	22 Participants n=7 Participants	32 Participants n=5 Participants
Region of Enrollment Czechia	2 Participants n=5 Participants	2 Participants n=7 Participants	4 Participants n=5 Participants
Region of Enrollment United Kingdom	13 Participants n=5 Participants	8 Participants n=7 Participants	21 Participants n=5 Participants
Region of Enrollment Switzerland	1 Participants n=5 Participants	0 Participants n=7 Participants	1 Participants n=5 Participants
Region of Enrollment Spain	8 Participants n=5 Participants	6 Participants n=7 Participants	14 Participants n=5 Participants
Region of Enrollment Canada	7 Participants n=5 Participants	6 Participants n=7 Participants	13 Participants n=5 Participants
Region of Enrollment Austria	3 Participants n=5 Participants	1 Participants n=7 Participants	4 Participants n=5 Participants
Region of Enrollment Netherlands	1 Participants n=5 Participants	0 Participants n=7 Participants	1 Participants n=5 Participants
Region of Enrollment Turkey	4 Participants n=5 Participants	5 Participants n=7 Participants	9 Participants n=5 Participants
Region of Enrollment Belgium	5 Participants n=5 Participants	3 Participants n=7 Participants	8 Participants n=5 Participants
Region of Enrollment Italy	6 Participants n=5 Participants	4 Participants n=7 Participants	10 Participants n=5 Participants
Region of Enrollment Israel	4 Participants n=5 Participants	2 Participants n=7 Participants	6 Participants n=5 Participants
Region of Enrollment Australia	1 Participants n=5 Participants	2 Participants n=7 Participants	3 Participants n=5 Participants
Region of Enrollment France	3 Participants n=5 Participants	9 Participants n=7 Participants	12 Participants n=5 Participants

PRIMARY outcome

Timeframe: Only data from the DB daily PE period (median = 5 days) up to the cut-off were used. The cut-off point was defined by whichever occured first: 1) 45 days of daily PE after start of study drug, 2) stop of daily PE, 3) stop of study drug (median = 34 days)

Population: Intent-to-treat (ITT) population (for the respective study period, i.e., DB treatment period)

Platelet count response was defined as initial platelet count ≥ 150,000/μL with subsequent stop of daily PE within 5 days. It refers to the first time both conditions, platelet count ≥ 150,000/μL and the stop of daily PE within 5 days, were met.

Outcome measures

Measure	Caplacizumab n=71 Participants Caplacizumab 10 mg once daily	Placebo n=73 Participants Placebo once daily
Time to Platelet Count Response	2.69 days Interval 1.89 to 2.83	2.88 days Interval 2.68 to 3.56

SECONDARY outcome

Timeframe: The study drug treatment period, a median (min, max) of 36 (2, 82) days. For both treatment groups, only events that occurred prior to a switch to open-label caplacizumab were evaluated for this analysis.

Population: ITT Population (for the respective study period, i.e., overall treatment period)

Number and percentage of subjects with TTP-related death, a recurrence of TTP, or at least one treatment-emergent major thromboembolic event during the study drug treatment period (i.e., first key secondary endpoint).

Outcome measures

Measure	Caplacizumab n=71 Participants Caplacizumab 10 mg once daily	Placebo n=73 Participants Placebo once daily
Number and Percentage of Subjects With TTP-Related Death, Recurrence of TTP, or a Major Thromboembolic Event During the Study Drug Treatment Period	9 Participants	36 Participants

SECONDARY outcome

Timeframe: The overall study period (covers both the overall treatment period and the follow-up period), a median (min, max) of 65 (2, 110) days.

Population: ITT Population (for the respective study period, i.e., overall study period)

Number and percentage of subjects with a recurrence of TTP during the Overall Study Period (i.e., including follow-up [FU]) (i.e., second key secondary endpoint).

Outcome measures

Measure	Caplacizumab n=71 Participants Caplacizumab 10 mg once daily	Placebo n=73 Participants Placebo once daily
Number and Percentage of Subjects With a Recurrence of TTP in the Overall Study Period	9 Participants	28 Participants

SECONDARY outcome

Timeframe: The study drug treatment period, a median (min, max) of 36 (2, 82) days.

Population: ITT Population (for the respective study period, i.e., overall treatment period)

Number and percentage of subjects with refractory TTP, defined as absence of platelet count doubling after 4 days of standard treatment, and lactate dehydrogenase (LDH) > upper limit of normal (ULN) (i.e., third key secondary endpoint).

Outcome measures

Measure	Caplacizumab n=71 Participants Caplacizumab 10 mg once daily	Placebo n=73 Participants Placebo once daily
Number and Percentage of Subjects With Refractory Disease	0 Participants	3 Participants

SECONDARY outcome

Timeframe: Overall study period, a median (min, max) of 65 (2, 110) days. For both treatment groups, normalizations occurring during the open-label period were not evaluated in this analysis.

Population: ITT Population (for the respective study period, i.e., overall study period) with biomarker level data available

Time to first normalization of LDH, cardiac troponin I (cTnI) and serum creatinine was defined as: first time of LDH ≤ ULN and cTnI ≤ ULN and serum creatinine ≤ ULN - time of first i.v. loading dose of study drug after randomization + 1 minute. Subjects in either initial treatment group who switched to open-label caplacizumab before having reached the endpoint were censored at time of switch.

Of note, the key secondary endpoints were hierarchically ordered to allow statistical testing for these endpoints at the same nominal significance level of 5% without adjustment, as long as the tests occurred in the pre-defined sequential order, and given that all null hypotheses tested for endpoints with a higher rank (including the primary endpoint) were rejected. No confirmatory testing was done for this fourth key secondary endpoint, as the statistical test was not significant for the proportion of subjects with refractory disease (i.e., the third key secondary endpoint).

Outcome measures

Measure	Caplacizumab n=65 Participants Caplacizumab 10 mg once daily	Placebo n=66 Participants Placebo once daily
Time to Normalization of Organ Damage Marker Levels	2.86 days Interval 1.93 to 3.86	3.36 days Interval 1.88 to 7.71

OTHER_PRE_SPECIFIED outcome

Timeframe: Overall study drug treatment period, a median (min, max) of 36 (2, 82) days.

Population: ITT Population (for the respective study period, i.e., overall treatment period)

The number of days of PE during the overall study drug treatment period, including the number of days of PE during the open-label study drug treatment period. Data were analyzed according to the initial treatment allocation (both before and after switch to open-label caplacizumab).

Outcome measures

Measure	Caplacizumab n=71 Participants Caplacizumab 10 mg once daily	Placebo n=73 Participants Placebo once daily
Number of Days of Plasma Exchange	5.8 days Standard Error 0.51	9.4 days Standard Error 0.81

OTHER_PRE_SPECIFIED outcome

Timeframe: Overall study drug treatment period, a median (min, max) of 36 (2, 82) days.

Population: ITT Population (for the respective study period, i.e., overall treatment period)

The total volume of PE during the overall study drug treatment period, including the total volume of PE during the open-label study drug treatment period. Data were analyzed according to the initial treatment allocation (both before and after switch to open-label caplacizumab).

Outcome measures

Measure	Caplacizumab n=71 Participants Caplacizumab 10 mg once daily	Placebo n=73 Participants Placebo once daily
Total Volume of Plasma Exchange	21.33 liter(s) Standard Error 1.619	35.93 liter(s) Standard Error 4.169

OTHER_PRE_SPECIFIED outcome

Timeframe: Overall study drug treatment period, a median (min, max) of 36 (2, 82) days.

Population: ITT Population (for the respective study period, i.e., overall treatment period)

The number of days in intensive care unit (ICU) during the overall study drug treatment period, including the number of days in ICU during the open-label study drug treatment period. Data were analyzed according to the initial treatment allocation (both before and after switch to open-label caplacizumab).

Outcome measures

Measure	Caplacizumab n=71 Participants Caplacizumab 10 mg once daily	Placebo n=73 Participants Placebo once daily
Number of Days in Intensive Care Unit	3.4 days Standard Error 0.40	9.7 days Standard Error 2.12

OTHER_PRE_SPECIFIED outcome

Timeframe: Overall study drug treatment period, a median (min, max) of 36 (2, 82) days.

Population: ITT Population (for the respective study period, i.e., overall treatment period)

The number of days in hospital during the overall study drug treatment period, including the number of days in hospital during the open-label study drug treatment period. Data were analyzed according to the initial treatment allocation (both before and after switch to open-label caplacizumab).

Outcome measures

Measure	Caplacizumab n=71 Participants Caplacizumab 10 mg once daily	Placebo n=73 Participants Placebo once daily
Number of Days in Hospital	9.9 days Standard Error 0.70	14.4 days Standard Error 1.22

Adverse Events

Double-blind Caplacizumab

Serious events: 28 serious events

Other events: 67 other events

Deaths: 1 deaths

Double-blind Placebo

Serious events: 39 serious events

Other events: 65 other events

Deaths: 3 deaths

Open-label Caplacizumab

Serious events: 7 serious events

Other events: 25 other events

Deaths: 0 deaths

Serious adverse events

Measure	Double-blind Caplacizumab n=71 participants at risk Caplacizumab 10 mg once daily	Double-blind Placebo n=73 participants at risk Placebo once daily	Open-label Caplacizumab n=28 participants at risk In case an exacerbation during the 30-day treatment period or a relapse during the treatment extension period occurred (first exacerbation or relapse), subjects were to receive open label caplacizumab 10 mg daily together with re-initiation of daily PE and optimized immunosuppressive treatment. Caplacizumab treatment schedule and visit schedule were the same as for the initial study drug treatment period (covering daily PE \[variable duration\] and 30-day post-daily PE period) and the possible treatment extension period.
General disorders Asthenia	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
General disorders Systemic inflammatory response syndrome	0.00% 0/71 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	1.4% 1/73 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Gastrointestinal disorders Gingival bleeding	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Gastrointestinal disorders Upper gastrointestinal hemorrhage	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	3.6% 1/28 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Gastrointestinal disorders Colitis	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Gastrointestinal disorders Gastric ulcer hemorrhage	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Gastrointestinal disorders Gastrointestinal necrosis	0.00% 0/71 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	1.4% 1/73 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Gastrointestinal disorders Hematemesis	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Gastrointestinal disorders Intestinal ischemia	0.00% 0/71 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	1.4% 1/73 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Gastrointestinal disorders Intestinal perforation	0.00% 0/71 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	1.4% 1/73 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Gastrointestinal disorders Small intestinal obstruction	0.00% 0/71 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	1.4% 1/73 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Nervous system disorders Headache	2.8% 2/71 • Number of events 2 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Nervous system disorders Seizure	0.00% 0/71 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	3.6% 1/28 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Nervous system disorders Cerebral ischemia	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Nervous system disorders Encephalopathy	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Nervous system disorders Hemorrhagic transformation stroke	0.00% 0/71 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	1.4% 1/73 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Nervous system disorders Hemiparesis	0.00% 0/71 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	1.4% 1/73 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Skin and subcutaneous tissue disorders Rash erythematosus	0.00% 0/71 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	3.6% 1/28 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Respiratory, thoracic and mediastinal disorders Epistaxis	5.6% 4/71 • Number of events 4 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Respiratory, thoracic and mediastinal disorders Dyspnoea	0.00% 0/71 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	3.6% 1/28 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Respiratory, thoracic and mediastinal disorders Hypoxia	0.00% 0/71 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	1.4% 1/73 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Respiratory, thoracic and mediastinal disorders Respiratory failure	0.00% 0/71 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	1.4% 1/73 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Respiratory, thoracic and mediastinal disorders Pulmonary embolism	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Blood and lymphatic system disorders Thrombotic thrombocytopenic purpura	12.7% 9/71 • Number of events 9 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	39.7% 29/73 • Number of events 29 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	14.3% 4/28 • Number of events 4 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Blood and lymphatic system disorders Thrombotic microangiopathy	0.00% 0/71 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	1.4% 1/73 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Infections and infestations Septic shock	0.00% 0/71 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	2.7% 2/73 • Number of events 2 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Infections and infestations Bacteremia	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Infections and infestations Device related sepsis	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Infections and infestations Diverticulitis	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Musculoskeletal and connective tissue disorders Pain in extremity	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Musculoskeletal and connective tissue disorders Arthropathy	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Injury, poisoning and procedural complications Anaphylactic transfusion reaction	0.00% 0/71 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	4.1% 3/73 • Number of events 3 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Injury, poisoning and procedural complications Subarachnoid hemorrhage	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Vascular disorders Deep vein thrombosis	0.00% 0/71 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	1.4% 1/73 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Vascular disorders Jugular vein thrombosis	0.00% 0/71 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	1.4% 1/73 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Cardiac disorders Myocardial infarction	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	1.4% 1/73 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Cardiac disorders Arteriospasm coronary	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Cardiac disorders Cardiac tamponade	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Cardiac disorders Cardiogenic shock	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Cardiac disorders Ventricular fibrillation	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Investigations Gamma-glutamyltransferase increased	0.00% 0/71 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	1.4% 1/73 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Investigations Platelet count decreased	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Reproductive system and breast disorders Menorrhagia	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Reproductive system and breast disorders Hemorrhagic ovarian cyst	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Hepatobiliary disorders Bile duct stone	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Hepatobiliary disorders Cholecystitis	0.00% 0/71 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	1.4% 1/73 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Hepatobiliary disorders Gallbladder necrosis	0.00% 0/71 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	1.4% 1/73 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Immune system disorders Serum sickness	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.

Other adverse events

Measure	Double-blind Caplacizumab n=71 participants at risk Caplacizumab 10 mg once daily	Double-blind Placebo n=73 participants at risk Placebo once daily	Open-label Caplacizumab n=28 participants at risk In case an exacerbation during the 30-day treatment period or a relapse during the treatment extension period occurred (first exacerbation or relapse), subjects were to receive open label caplacizumab 10 mg daily together with re-initiation of daily PE and optimized immunosuppressive treatment. Caplacizumab treatment schedule and visit schedule were the same as for the initial study drug treatment period (covering daily PE \[variable duration\] and 30-day post-daily PE period) and the possible treatment extension period.
General disorders Catheter site hemorrhage	7.0% 5/71 • Number of events 6 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	6.8% 5/73 • Number of events 5 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	28.6% 8/28 • Number of events 10 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
General disorders Fatigue	14.1% 10/71 • Number of events 10 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	8.2% 6/73 • Number of events 6 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	7.1% 2/28 • Number of events 2 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
General disorders Pyrexia	14.1% 10/71 • Number of events 12 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	8.2% 6/73 • Number of events 6 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	3.6% 1/28 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
General disorders Oedema peripheral	5.6% 4/71 • Number of events 4 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	9.6% 7/73 • Number of events 8 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	3.6% 1/28 • Number of events 2 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
General disorders Asthenia	2.8% 2/71 • Number of events 3 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	5.5% 4/73 • Number of events 4 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	7.1% 2/28 • Number of events 3 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
General disorders Chest pain	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	6.8% 5/73 • Number of events 6 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	3.6% 1/28 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
General disorders Catheter site pain	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	6.8% 5/73 • Number of events 5 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
General disorders Injection site pain	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	5.5% 4/73 • Number of events 4 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	3.6% 1/28 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
General disorders Injection site hematoma	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	4.1% 3/73 • Number of events 4 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	7.1% 2/28 • Number of events 2 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
General disorders Pain	5.6% 4/71 • Number of events 7 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	1.4% 1/73 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
General disorders Injection site erythema	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	1.4% 1/73 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	7.1% 2/28 • Number of events 4 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
General disorders Injection site pruritus	2.8% 2/71 • Number of events 2 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	7.1% 2/28 • Number of events 2 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
General disorders Injection site reaction	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	7.1% 2/28 • Number of events 2 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
General disorders Face oedema	0.00% 0/71 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	7.1% 2/28 • Number of events 2 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Gastrointestinal disorders Nausea	14.1% 10/71 • Number of events 12 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	9.6% 7/73 • Number of events 8 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	7.1% 2/28 • Number of events 2 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Gastrointestinal disorders Gingival bleeding	16.9% 12/71 • Number of events 13 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	1.4% 1/73 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	14.3% 4/28 • Number of events 5 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Gastrointestinal disorders Constipation	9.9% 7/71 • Number of events 7 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	6.8% 5/73 • Number of events 6 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	14.3% 4/28 • Number of events 6 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Gastrointestinal disorders Diarrhoea	9.9% 7/71 • Number of events 7 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	6.8% 5/73 • Number of events 5 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	14.3% 4/28 • Number of events 7 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Gastrointestinal disorders Abdominal pain	7.0% 5/71 • Number of events 5 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	5.5% 4/73 • Number of events 4 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	7.1% 2/28 • Number of events 4 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Gastrointestinal disorders Vomiting	4.2% 3/71 • Number of events 3 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	5.5% 4/73 • Number of events 5 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	7.1% 2/28 • Number of events 2 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Gastrointestinal disorders Abdominal pain upper	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	1.4% 1/73 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	14.3% 4/28 • Number of events 8 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Gastrointestinal disorders Dyspepsia	2.8% 2/71 • Number of events 2 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	1.4% 1/73 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	7.1% 2/28 • Number of events 2 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Gastrointestinal disorders Hematochezia	2.8% 2/71 • Number of events 5 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	7.1% 2/28 • Number of events 2 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Gastrointestinal disorders Lip hemorrhage	0.00% 0/71 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	7.1% 2/28 • Number of events 2 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Nervous system disorders Headache	19.7% 14/71 • Number of events 18 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	8.2% 6/73 • Number of events 10 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	21.4% 6/28 • Number of events 6 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Nervous system disorders Dizziness	9.9% 7/71 • Number of events 8 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	11.0% 8/73 • Number of events 8 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	7.1% 2/28 • Number of events 3 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Nervous system disorders Paresthesia	11.3% 8/71 • Number of events 9 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	8.2% 6/73 • Number of events 6 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Skin and subcutaneous tissue disorders Urticaria	16.9% 12/71 • Number of events 14 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	6.8% 5/73 • Number of events 8 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	3.6% 1/28 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Skin and subcutaneous tissue disorders Rash	7.0% 5/71 • Number of events 6 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	12.3% 9/73 • Number of events 11 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	14.3% 4/28 • Number of events 4 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Skin and subcutaneous tissue disorders Pruritus	7.0% 5/71 • Number of events 6 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	8.2% 6/73 • Number of events 6 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	7.1% 2/28 • Number of events 4 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Skin and subcutaneous tissue disorders Petechiae	5.6% 4/71 • Number of events 4 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	6.8% 5/73 • Number of events 5 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	10.7% 3/28 • Number of events 5 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Skin and subcutaneous tissue disorders Ecchymosis	2.8% 2/71 • Number of events 2 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	5.5% 4/73 • Number of events 4 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	10.7% 3/28 • Number of events 3 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Respiratory, thoracic and mediastinal disorders Epistaxis	29.6% 21/71 • Number of events 32 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	2.7% 2/73 • Number of events 2 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	17.9% 5/28 • Number of events 5 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Respiratory, thoracic and mediastinal disorders Dyspnoea	9.9% 7/71 • Number of events 10 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	2.7% 2/73 • Number of events 2 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	7.1% 2/28 • Number of events 2 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Blood and lymphatic system disorders Anemia	5.6% 4/71 • Number of events 4 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	8.2% 6/73 • Number of events 9 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	14.3% 4/28 • Number of events 8 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Infections and infestations Urinary tract infection	5.6% 4/71 • Number of events 4 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	5.5% 4/73 • Number of events 4 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Infections and infestations Viral upper respiratory tract infection	5.6% 4/71 • Number of events 4 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Musculoskeletal and connective tissue disorders Pain in extremity	5.6% 4/71 • Number of events 5 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	8.2% 6/73 • Number of events 7 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Musculoskeletal and connective tissue disorders Arthralgia	5.6% 4/71 • Number of events 4 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	4.1% 3/73 • Number of events 3 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	10.7% 3/28 • Number of events 4 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Musculoskeletal and connective tissue disorders Back pain	7.0% 5/71 • Number of events 5 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	4.1% 3/73 • Number of events 3 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	3.6% 1/28 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Musculoskeletal and connective tissue disorders Muscular weakness	5.6% 4/71 • Number of events 5 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	2.7% 2/73 • Number of events 2 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Musculoskeletal and connective tissue disorders Myalgia	2.8% 2/71 • Number of events 2 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	1.4% 1/73 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	7.1% 2/28 • Number of events 3 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Metabolism and nutrition disorders Hypokalemia	8.5% 6/71 • Number of events 6 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	19.2% 14/73 • Number of events 15 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	7.1% 2/28 • Number of events 2 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Metabolism and nutrition disorders Hyperglycemia	5.6% 4/71 • Number of events 5 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	5.5% 4/73 • Number of events 4 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	3.6% 1/28 • Number of events 2 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Metabolism and nutrition disorders Hypocalcemia	1.4% 1/71 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	6.8% 5/73 • Number of events 8 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	7.1% 2/28 • Number of events 3 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Psychiatric disorders Insomnia	8.5% 6/71 • Number of events 7 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	11.0% 8/73 • Number of events 8 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Psychiatric disorders Anxiety	5.6% 4/71 • Number of events 4 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	8.2% 6/73 • Number of events 6 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	3.6% 1/28 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Psychiatric disorders Agitation	7.0% 5/71 • Number of events 6 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	5.5% 4/73 • Number of events 4 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Injury, poisoning and procedural complications Contusion	7.0% 5/71 • Number of events 7 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	13.7% 10/73 • Number of events 24 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	7.1% 2/28 • Number of events 10 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Vascular disorders Hypertension	5.6% 4/71 • Number of events 4 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	11.0% 8/73 • Number of events 8 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	3.6% 1/28 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Vascular disorders Hematoma	4.2% 3/71 • Number of events 7 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	2.7% 2/73 • Number of events 2 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	7.1% 2/28 • Number of events 2 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Vascular disorders Hypotension	5.6% 4/71 • Number of events 4 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	2.7% 2/73 • Number of events 3 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	3.6% 1/28 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Vascular disorders Hot flush	0.00% 0/71 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/73 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	7.1% 2/28 • Number of events 2 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Cardiac disorders Sinus tachycardia	5.6% 4/71 • Number of events 4 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	4.1% 3/73 • Number of events 5 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	3.6% 1/28 • Number of events 4 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Cardiac disorders Tachycardia	2.8% 2/71 • Number of events 2 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	5.5% 4/73 • Number of events 4 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	3.6% 1/28 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Investigations Hepatic enzyme increased	0.00% 0/71 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	1.4% 1/73 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	7.1% 2/28 • Number of events 2 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Renal and urinary disorders Hematuria	7.0% 5/71 • Number of events 7 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	2.7% 2/73 • Number of events 2 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	7.1% 2/28 • Number of events 2 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Reproductive system and breast disorders Vaginal hemorrhage	5.6% 4/71 • Number of events 5 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	2.7% 2/73 • Number of events 2 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	3.6% 1/28 • Number of events 1 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.
Eye disorders Vision blurred	7.0% 5/71 • Number of events 6 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	6.8% 5/73 • Number of events 5 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.	0.00% 0/28 • From time of first study drug administration until the subject's study completion/discontinuation date, a median (min, max) of 65 (2, 110) days. Double-Blind Caplacizumab/Double-Blind placebo groups (subjects randomized to caplacizumab/placebo, respectively): adverse events (AEs) starting in DB or FU Periods for subjects with no OL Period. Only AEs starting in the DB Period for subjects with an OL Period. OL Caplacizumab group (all subjects with OL Period): AEs starting in the OL or FU Periods. The 3 subjects who died in the placebo group died during treatment and the subject who died in the caplacizumab group died during follow-up.

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Results disclosure agreements

• Principal investigator is a sponsor employee Publication of any results from this study will be according to the principles of the Declaration of Helsinki, in particular point 30, and will require prior review and written agreement of the Sponsor.
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Restriction type: OTHER