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Evaluate the PK Efficacy Safety and Tolerability of Pegcetacoplan in Patients With Thrombotic Microangiopathy

Phase 2
Completed
Conditions
Transplant-Associated Thrombotic Microangiopathy
Interventions
Registration Number
NCT05148299
Lead Sponsor
Swedish Orphan Biovitrum
Brief Summary

The purpose of the study is to assess PK, Pharmacodynamics (PD), Efficacy and safety of pegcetacoplan in patients with TA-TMA after HSCT.

Detailed Description

This is a pilot study and the sample size is based on practical rather than statistical aspects.

A total of 12 patients will be included and treated in the study. With 12 patients included, it is estimated that 9 patients will complete at least 4 weeks of treatment, which is deemed sufficient to characterize the PK of pegcetacoplan in patients with TA-TMA to an appropriate precision. In addition, 12 patients will provide a 72 % probability to observe a response rate of at least 8 responders of the 12 patients recruited (assuming the true response rates is 70 %).

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
12
Inclusion Criteria
  1. Male and female patients aged ≥ 18 years at the time of informed consent form (ICF) signature.

  2. Received allogeneic HSCT.

  3. Diagnosis of TA-TMA established, as per laboratory markers indicating TMA.

  4. Have a diagnosis of TA-TMA that persists despite initial management of any triggering condition.

  5. Have rUPCR ≥ 1 mg/mg.

  6. Women of childbearing potential, defined as any women who have experienced menarche and who are NOT permanently sterile or postmenopausal, must have a negative serum pregnancy test at screening and agree to use protocol-defined methods of contraception for the duration of the study and 8 weeks after their last IMP dose.

    Note: Postmenopausal is defined as having had 12 consecutive months with no menses without an alternative medical cause.

  7. Men must agree to the following for the duration of the study and 8 weeks after their last dose of IMP:

    1. Avoid fathering a child.
    2. Use protocol-defined methods of contraception.
    3. Refrain from donating sperm.
  8. Patient and/or legally authorized representative must be capable of giving signed informed consent, which includes compliance with the requirements and restrictions listed in the ICF.

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Exclusion Criteria
  1. Positive direct Coombs test.
  2. Known familial or acquired ADAMTS13 deficiency.
  3. Known Shiga toxin-related hemolytic uremic syndrome.
  4. Known bone marrow or graft failure.
  5. Diagnosis of disseminated intravascular coagulation.
  6. Diagnosis of veno-occlusive disease (VOD).
  7. Active GI bleeding (hematemesis or hematochezia) at baseline.
  8. Body weight < 30 kg and > 100 kg.
  9. Uncontrolled systemic bacterial or fungal infection, presence or suspicion of sepsis.
  10. Previously or currently treated with a complement inhibitor (approved or investigational).
  11. Pregnancy or breastfeeding.
  12. Positive human immunodeficiency virus antibody at screening or documented in pre-HSCT medical record.
  13. Hepatitis C virus detectable by polymerase chain reaction at screening or documented in pre-HSCT medical record.
  14. Chronic inactive hepatitis B virus with viral loads > 1000 IU/mL (> 5000 copies/mL) at screening or documented in pre-HSCT medical record. Eligible patients who are chronic active carriers (≤ 1000 IU/mL) must receive prophylactic antiviral treatment (e.g., entecavir, tenofovir, lamivudine) according to local country guidelines.
  15. Known or suspected hereditary fructose intolerance.
  16. Hypersensitivity to pegcetacoplan or any of its excipients.
  17. Inability to cooperate with study procedures or any condition that, in the opinion of the investigator, could increase the patient's risk by participating in the study or confound the outcome of the study.
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Study & Design

Study Type
INTERVENTIONAL
Study Design
SINGLE_GROUP
Arm && Interventions
GroupInterventionDescription
PegcetacoplanPegcetacoplansterile solution in stoppered glass vial given as 1080 mg infusion 3 times weekly for 12 weeks.
Primary Outcome Measures
NameTimeMethod
Pegcetacoplan PK parameter TmaxWeek 24

Time of maximum measured serum concentration

Pegcetacoplan PK parameter CmaxWeek 24

Maximum observed serum concentration

Pegcetacoplan PK parameter CtroughWeek 24

Observed serum concentration predose

Pegcetacoplan PK parameter AUC0-tauWeek 24

Area under the concentration-time curve over the dosing interval

Secondary Outcome Measures
NameTimeMethod
Absolute levels, change from baseline, and percent change in sC5b-9Week 24

Absolute levels, change from baseline, and percent change from baseline to Week 24 in biomarker of complement activation sC5b-9

Time to clinical response.From treatment start to first documentation of attainment of a clinical response

Both clinical response sustained at week 24 and clinical response at any time during the study will be assessed.

Duration of clinical response.From the first observed clinical response until the response criteria is no longer fulfilled or until end of study

Duration of clinical response sustained at week 24

Duration of TMA response.From the first observed TMA response until the response criteria is no longer fulfilled or until end of study

Duration of TMA response sustained at week 24

TA-TMA relapse at Week 24.Week 24

A participant will be declared as relapsing upon appearance of laboratory markers of TMA

Number of participants reaching clinical response at week 12Week 12

Number of participants reaching clinical response at week 12

Number of participants reaching TMA response at week 12Week 12

Number of participants reaching TMA response at week 12

Number of participants reaching clinical response at week 24Week 24

A participant will be declared as reaching a clinical response upon improvement in laboratory markers of TMA and resolution of TMA clinical symptoms

Number of participants reaching TMA response at week 24Week 24

A participant will be declared as reaching TMA response upon improvement in laboratory markers of TMA

Overall survivalWeek 24 from treatment start

Survival

Time to TMA response.From treatment start to first documentation of attainment of a TMA response

Both TMA response sustained at week 24 and TMA response at any time during the study will be assessed.

Trial Locations

Locations (14)

University General Hospital "Attikon"

🇬🇷

Athens, Chaidari, Greece

CHU de Saint-Etienne

🇫🇷

Saint-Priest-en-Jarez, France

General Hospital of Thessaloniki "G. Papanikolaou", Hematology Department - BMT Unit

🇬🇷

Thessaloníki, Greece

Hospital San Giuseppe Moscati

🇮🇹

Avellino, Italy

ASST Monza - S. Gerardo Hospital

🇮🇹

Monza, Italy

Big Metropolitan Hospital Niguarda Regional Health Authority

🇮🇹

Milan, Italy

University Polyclinic Foundation "Agostino Gemelli" - IRCCS

🇮🇹

Roma, Italy

City of Hope

🇺🇸

Duarte, California, United States

Archet 1 hospital, Department of Clinical Hematology

🇫🇷

Nice, France

Mayo Clinic - Rochester

🇺🇸

Rochester, Minnesota, United States

Saint-Louis Hospital

🇫🇷

Paris, France

General Hospital of Athens "Evangelismos"

🇬🇷

Athens, Greece

United Hospitals Villa Sofia Cervello

🇮🇹

Palermo, Italy

University Hospital Puerta de Hierro Majadahonda

🇪🇸

Madrid, Spain

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