Study to Evaluate the Efficacy and Safety of APL-2 in Patients With Paroxysmal Nocturnal Hemoglobinuria (PNH)

NCT ID: NCT03500549

Last Updated: 2022-03-25

Study Results

Results available

Outcome measurements, participant flow, baseline characteristics, and adverse events have been published for this study.

View full results

Basic Information

Get a concise snapshot of the trial, including recruitment status, study phase, enrollment targets, and key timeline milestones.

Recruitment Status

COMPLETED

Clinical Phase

PHASE3

Total Enrollment

80 participants

Study Classification

INTERVENTIONAL

Study Start Date

2018-06-14

Study Completion Date

2020-08-13

Brief Summary

Review the sponsor-provided synopsis that highlights what the study is about and why it is being conducted.

Evaluation of the Efficacy and Safety of APL-2 in Patients with Paroxysmal Nocturnal Hemoglobinuria

Detailed Description

Dive into the extended narrative that explains the scientific background, objectives, and procedures in greater depth.

Conditions

See the medical conditions and disease areas that this research is targeting or investigating.

Paroxysmal Nocturnal Hemoglobinuria

Study Design

Understand how the trial is structured, including allocation methods, masking strategies, primary purpose, and other design elements.

Allocation Method

RANDOMIZED

Intervention Model

CROSSOVER

Primary Study Purpose

TREATMENT

Blinding Strategy

NONE

Study Groups

Review each arm or cohort in the study, along with the interventions and objectives associated with them.

Pegcetacoplan

1080 mg pegcetacoplan administered subcutaneously twice-weekly or every three days.

Group Type EXPERIMENTAL

Pegcetacoplan

Intervention Type DRUG

Complement (C3) Inhibitor

Soliris

Intervention Type DRUG

Complement (C5) Inhibitor

Eculizumab

Complement (C5) Inhibitor.

Group Type ACTIVE_COMPARATOR

Pegcetacoplan

Intervention Type DRUG

Complement (C3) Inhibitor

Soliris

Intervention Type DRUG

Complement (C5) Inhibitor

Interventions

Learn about the drugs, procedures, or behavioral strategies being tested and how they are applied within this trial.

Pegcetacoplan

Complement (C3) Inhibitor

Intervention Type DRUG

Soliris

Complement (C5) Inhibitor

Intervention Type DRUG

Other Intervention Names

Discover alternative or legacy names that may be used to describe the listed interventions across different sources.

APL-2

Eligibility Criteria

Check the participation requirements, including inclusion and exclusion rules, age limits, and whether healthy volunteers are accepted.

Inclusion Criteria

* At least 18 years of age
* Primary diagnosis of PNH confirmed by high-sensitivity flow cytometry
* On treatment with eculizumab. Dose of eculizumab must have been stable for at least 3 months prior to the Screening Visit
* Hb \<10.5 g/dL at the Screening Visit
* Absolute reticulocyte count \> 1.0x ULN at the Screening Visit
* Platelet count of \>50,000/mm3 at the Screening Visit
* Absolute neutrophil count \>500/mm3 at the Screening Visit
* Vaccination against Neisseria meningitides types A, C, W, Y and B, Streptococcus pneumoniae and Haemophilus influenzae Type B (Hib) either within 2 years prior to Day 1 dosing, or within 14 days after starting treatment with APL-2. Unless documented evidence exists that subjects are non-responders to vaccination as evidenced by titers or display titer levels within acceptable local limits
* Women of child-bearing potential (WOCBP) must have a negative pregnancy test at the Screening and Day -28 Visit (Run-in Period) and must agree to use protocol defined methods of contraception for the duration of the study and 90 days after their last dose of study drug
* Males must agree to use protocol defined methods of contraception and agree to refrain from donating sperm for the duration of the study and 90 days after their last dose of study drug
* Willing and able to give informed consent
* Willing and able to self-administer APL-2 (administration by caregiver will be allowed)
* Have a body mass index (BMI) ≤35.0 kg/m2

Exclusion Criteria

* Active bacterial infection that has not resolved within 14 week of Day -28 (first dose of APL-2)
* Receiving iron, folic acid, vitamin B12 and EPO, unless the dose is stable, in the 4 weeks prior to Screening
* Hereditary complement deficiency
* History of bone marrow transplantation
* History or presence of hypersensitivity or idiosyncratic reaction to compounds related to the investigational product or SC administration
* Participation in any other investigational drug trial or exposure to other investigational agent within 30 days or 5 half-lives (whichever is longer)
* Currently breast-feeding women
* Inability to cooperate or any condition that, in the opinion of the investigator, could increase the subject's risk of participating in the study or confound the outcome of the study

This study includes cardiac safety evaluations. The following cardiac eligibility criteria are necessary to avoid confounding the cardiac safety outcomes:

* History or family history of Long QT Syndrome or torsade de pointes, unexplained syncope, syncope from an uncorrected cardiac etiology, or family history of sudden death
* Myocardial infarction, CABG, coronary or cerebral artery stenting and /or angioplasty, stroke, cardiac surgery, or hospitalization for congestive heart failure within 3 months or greater than Class 2 Angina Pectoris or NYHA Heart Failure Class \>2
* QTcF \> 470 ms, PR \> 280 ms
* Mobitz II 2nd degree AV Block, 2:1 AV Block, High Grade AV Block, or Complete Heart Block unless the patient has an implanted pacemaker or implantable cardiac defibrillator (ICD) with backup pacing capabilities
* Receiving Class 1 or Class 3 antiarrhythmic agents, or arsenic, methadone, ondansetron or pentamidine at screening
* Receiving any other QTc-prolonging drugs (see Appendix 4 in Section 19.4), at a stable dose for less than 3 weeks prior to dosing
* Receiving prophylactic ciprofloxacin, erythromycin or azithromycin for less than one week prior to the first dose of study medication (must have a repeat screening ECG after one week of prophylactic antibiotics with QTcF \< 470 ms)
Minimum Eligible Age

18 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

Meet the organizations funding or collaborating on the study and learn about their roles.

Apellis Pharmaceuticals, Inc.

INDUSTRY

Sponsor Role lead

Responsible Party

Identify the individual or organization who holds primary responsibility for the study information submitted to regulators.

Responsibility Role SPONSOR

Locations

Explore where the study is taking place and check the recruitment status at each participating site.

Samsung Medical Center

Seoul, , South Korea

Site Status

Soonchunhyang University Bucheon Hospital

Bucheon-si, , South Korea

Site Status

Chungnam National University Hospital

Daejeon, , South Korea

Site Status

University of Southern California

Los Angeles, California, United States

Site Status

Sarcoma Oncology Research Center

Santa Monica, California, United States

Site Status

Denver Health

Denver, Colorado, United States

Site Status

Georgetown University Hospital

Washington D.C., District of Columbia, United States

Site Status

Cancer Specialists of North Florida

Jacksonville, Florida, United States

Site Status

Lakes Research

Miami Lakes, Florida, United States

Site Status

Mid Florida Hematology and Oncology

Orange City, Florida, United States

Site Status

Winship Cancer Institute of Emory University

Atlanta, Georgia, United States

Site Status

Northwestern University

Chicago, Illinois, United States

Site Status

Investigative Clinical Research of Indiana

Indianapolis, Indiana, United States

Site Status

Cancer & Hematology Centers of Western Michigan

Grand Rapids, Michigan, United States

Site Status

Roswell Park Cancer Institute

Buffalo, New York, United States

Site Status

New York Cancer & Blood Specialists

East Setauket, New York, United States

Site Status

New York Cancer & Blood Specialists

The Bronx, New York, United States

Site Status

Duke University Medical Center

Durham, North Carolina, United States

Site Status

Good Samaritan Hospital

Corvallis, Oregon, United States

Site Status

University of Tennessee Medical Center

Knoxville, Tennessee, United States

Site Status

Baptist Cancer Center

Memphis, Tennessee, United States

Site Status

HOPE Cancer Center of East Texas

Tyler, Texas, United States

Site Status

Royal Melbourne Hospital

Melbourne, Victoria, Australia

Site Status

Cliniques Universitaires Saint-Luc

Woluwe-Saint-Lambert, Vlaams-Brabant, Belgium

Site Status

AZ Delta Campus Wilgenstraat

Roeselare, West-Vlaanderen, Belgium

Site Status

University of Alberta

Edmonton, Alberta, Canada

Site Status

Toronto General Hospital

Toronto, Ontario, Canada

Site Status

Centre Hospitalier Annecy Genevois

Annecy, , France

Site Status

Centre Hospitalier William Morey

Chalon-sur-Saône, , France

Site Status

Centre Hospitalier Universitaire de Lille

Lille, , France

Site Status

Institut Paoli-Calmettes Marseille

Marseille, , France

Site Status

Hôpital Saint-Louis

Paris, , France

Site Status

Centre Hospitalier Lyon Sud

Pierre-Bénite, , France

Site Status

Centre Hospitalier de Saint-Quentin

Saint-Quentin, , France

Site Status

Institut Universitaire du Cancer Toulouse - Oncopole

Toulouse, , France

Site Status

Universitätsklinikum Ulm

Ulm, Baden-Wurttemberg, Germany

Site Status

Uniklinik RWTH Aachen

Aachen, North Rhine-Westphalia, Germany

Site Status

Universitätsklinikum Essen

Essen, North Rhine-Westphalia, Germany

Site Status

Universitätsklinikum Hamburg Eppendorf

Hamburg, , Germany

Site Status

Japanese Red Cross Nagoya Daiichi Hospital

Nagoya, Aichi-ken, Japan

Site Status

Japanese Red Cross Nagoya Daini Hospital

Showa-ku, Aichi-ken, Japan

Site Status

University of Tsukuba Hospital

Tsukuba, Ibaraki, Japan

Site Status

Shinshu University Hospital

Matsumoto, Nagano, Japan

Site Status

Okayama University Hospital

Okayama, Okayama-ken, Japan

Site Status

Juntendo University Hospital

Bunkyo-ku, Tokyo, Japan

Site Status

NTT Medical Center Tokyo

Shinagawa-ku, Tokyo, Japan

Site Status

Kinan Hospital

Tanabe, Wakayama, Japan

Site Status

Pavlov First Saint Petersburg State Medical University of Russian Ministry of Health

Saint Petersburg, , Russia

Site Status

Pavlov First Saint Petersburg State Medical University

Saint Petersburg, , Russia

Site Status

Institution of Health Care of Tyumen Region

Tyumen, , Russia

Site Status

Hospital Universitario de Gran Canaria Dr. Negrín

Las Palmas de Gran Canaria, , Spain

Site Status

Hospital Universitario Politécnico La Fe

Valencia, , Spain

Site Status

St. James' Institute of Oncology, Leeds Teaching Hospitals

Leeds, , United Kingdom

Site Status

Countries

Review the countries where the study has at least one active or historical site.

United States Australia Belgium Canada France Germany Japan Russia South Korea Spain United Kingdom

References

Explore related publications, articles, or registry entries linked to this study.

de Castro C, Kelly RJ, Griffin M, Patriquin CJ, Mulherin B, Hochsmann B, Selvaratnam V, Wong RSM, Hillmen P, Horneff R, Uchendu UO, Zhang Y, Surova E, Szamosi J, de Latour RP. Efficacy and Safety Maintained up to 3 Years in Adults with Paroxysmal Nocturnal Hemoglobinuria Receiving Pegcetacoplan. Adv Ther. 2025 Sep;42(9):4641-4658. doi: 10.1007/s12325-025-03310-8. Epub 2025 Jul 28.

Reference Type DERIVED
PMID: 40720060 (View on PubMed)

de Latour RP, Kulasekararaj A, Dingli D, Wilson K, Wojciechowski P, Hakimi Z, Nazir J, Czech B, Hillmen P, Szer J. Anchored Indirect Treatment Comparison Finds Comparable Effects of Pegcetacoplan and Iptacopan in Paroxysmal Nocturnal Haemoglobinuria. Eur J Haematol. 2025 Aug;115(2):125-133. doi: 10.1111/ejh.14422. Epub 2025 Apr 25.

Reference Type DERIVED
PMID: 40285403 (View on PubMed)

Panse J, Daguindau N, Okuyama S, Peffault de Latour R, Schafhausen P, Straetmans N, Al-Adhami M, Persson E, Wong RSM. Improvements in hematologic markers and decreases in fatigue with pegcetacoplan for patients with paroxysmal nocturnal hemoglobinuria and mild or moderate anemia (hemoglobin >/=10 g/dL) who had received eculizumab or were naive to complement inhibitors. PLoS One. 2024 Jul 29;19(7):e0306407. doi: 10.1371/journal.pone.0306407. eCollection 2024.

Reference Type DERIVED
PMID: 39079163 (View on PubMed)

Mulherin BP, Yeh M, Al-Adhami M, Dingli D. Normalization of Hemoglobin, Lactate Dehydrogenase, and Fatigue in Patients with Paroxysmal Nocturnal Hemoglobinuria Treated with Pegcetacoplan. Drugs R D. 2024 Jun;24(2):169-177. doi: 10.1007/s40268-024-00463-9. Epub 2024 May 10.

Reference Type DERIVED
PMID: 38727860 (View on PubMed)

Peffault de Latour R, Griffin M, Kelly RJ, Szer J, de Castro C, Horneff R, Tan L, Yeh M, Panse J. Hemolysis events in the phase 3 PEGASUS study of pegcetacoplan in patients with paroxysmal nocturnal hemoglobinuria. Blood Adv. 2024 Jun 11;8(11):2718-2725. doi: 10.1182/bloodadvances.2024012672.

Reference Type DERIVED
PMID: 38593241 (View on PubMed)

Sharma V, Koprivnikar J, Drago K, Savage J, Bachelor A. Injection Site Reactions with Long-Term Pegcetacoplan Use in Patients with Paroxysmal Nocturnal Hemoglobinuria: A Brief Report. Adv Ther. 2023 Nov;40(11):5115-5129. doi: 10.1007/s12325-023-02653-4. Epub 2023 Sep 14.

Reference Type DERIVED
PMID: 37707673 (View on PubMed)

de Latour RP, Szer J, Weitz IC, Roth A, Hochsmann B, Panse J, Usuki K, Griffin M, Kiladjian JJ, de Castro CM, Nishimori H, Ajayi T, Al-Adhami M, Deschatelets P, Francois C, Grossi F, Risitano AM, Hillmen P. Pegcetacoplan versus eculizumab in patients with paroxysmal nocturnal haemoglobinuria (PEGASUS): 48-week follow-up of a randomised, open-label, phase 3, active-comparator, controlled trial. Lancet Haematol. 2022 Sep;9(9):e648-e659. doi: 10.1016/S2352-3026(22)00210-1.

Reference Type DERIVED
PMID: 36055332 (View on PubMed)

Cella D, Sarda SP, Hsieh R, Fishman J, Hakimi Z, Hoffman K, Al-Adhami M, Nazir J, Cutts K, Lenderking WR. Changes in hemoglobin and clinical outcomes drive improvements in fatigue, quality of life, and physical function in patients with paroxysmal nocturnal hemoglobinuria: post hoc analyses from the phase III PEGASUS study. Ann Hematol. 2022 Sep;101(9):1905-1914. doi: 10.1007/s00277-022-04887-8. Epub 2022 Jul 23.

Reference Type DERIVED
PMID: 35869984 (View on PubMed)

Hakimi Z, Wilson K, McAughey E, Pochopien M, Wojciechowski P, Toumi M, Knight C, Sarda SP, Patel N, Wiseman C, de Castro NP, Nazir J, Kelly RJ. The cost-effectiveness, of pegcetacoplan compared with ravulizumab for the treatment of paroxysmal nocturnal hemoglobinuria, in a UK setting. J Comp Eff Res. 2022 Sep;11(13):969-985. doi: 10.2217/cer-2022-0076. Epub 2022 Jul 7.

Reference Type DERIVED
PMID: 35796199 (View on PubMed)

Bhak RH, Mody-Patel N, Baver SB, Kunzweiler C, Yee CW, Sundaresan S, Swartz N, Duh MS, Krishnan S, Sarda SP. Comparative effectiveness of pegcetacoplan versus ravulizumab in patients with paroxysmal nocturnal hemoglobinuria previously treated with eculizumab: a matching-adjusted indirect comparison. Curr Med Res Opin. 2021 Nov;37(11):1913-1923. doi: 10.1080/03007995.2021.1971182. Epub 2021 Sep 3.

Reference Type DERIVED
PMID: 34445916 (View on PubMed)

Hillmen P, Szer J, Weitz I, Roth A, Hochsmann B, Panse J, Usuki K, Griffin M, Kiladjian JJ, de Castro C, Nishimori H, Tan L, Hamdani M, Deschatelets P, Francois C, Grossi F, Ajayi T, Risitano A, Peffault de Latour R. Pegcetacoplan versus Eculizumab in Paroxysmal Nocturnal Hemoglobinuria. N Engl J Med. 2021 Mar 18;384(11):1028-1037. doi: 10.1056/NEJMoa2029073.

Reference Type DERIVED
PMID: 33730455 (View on PubMed)

Provided Documents

Download supplemental materials such as informed consent forms, study protocols, or participant manuals.

Document Type: Study Protocol

View Document

Document Type: Statistical Analysis Plan

View Document

Other Identifiers

Review additional registry numbers or institutional identifiers associated with this trial.

APL2-302

Identifier Type: -

Identifier Source: org_study_id

More Related Trials

Additional clinical trials that may be relevant based on similarity analysis.

A Renal Impairment Study for PF-04965842
NCT03660241 COMPLETED PHASE1