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Pozelimab and Cemdisiran Combination Therapy in Adult Participants With Paroxysmal Nocturnal Hemoglobinuria Who Switch From Eculizumab Therapy

Phase 2
Completed
Conditions
Paroxysmal Nocturnal Hemoglobinuria
Interventions
Registration Number
NCT04888507
Lead Sponsor
Regeneron Pharmaceuticals
Brief Summary

The primary objective of the study is to evaluate the safety and tolerability of pozelimab and cemdisiran combination therapy in participants with PNH who switch from eculizumab therapy

The secondary objectives of the study are:

* To evaluate the effect of the combination treatment on the following parameters of intravascular hemolysis: lactate dehydrogenase (LDH) control, breakthrough hemolysis, and inhibition of CH50

* To evaluate the effect of the combination treatment on the stability of LDH during the transition period from eculizumab monotherapy to combination with pozelimab and cemdisiran

* To evaluate the effect of the combination treatment on red blood cell (RBC) transfusion requirements

* To evaluate the effect of the combination treatment on hemoglobin levels

* To evaluate the effect of the combination treatment on clinical outcome assessments (COAs) measuring fatigue and health related quality of life (HRQoL)

* To assess the concentrations of total pozelimab and eculizumab in serum; and total cemdisiran and C5 protein in plasma

* To assess the immunogenicity of pozelimab and cemdisiran

* To assess safety after dose intensification

* To evaluate the long-term safety and efficacy of the combination treatment in an optional open-label extension period (OLEP)

Detailed Description

Not available

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
6
Inclusion Criteria
  1. Diagnosis of paroxysmal nocturnal hemoglobinuria confirmed by a history of high-sensitivity flow cytometry from prior testing
  2. Treated with stable (ie, no change in dose or frequency) eculizumab therapy at the labeled dosing regimen or a higher dose and/or more frequently administered than labeled for at least 12 weeks prior to screening visit

Key

Exclusion Criteria
  1. History of bone marrow transplantation or receipt of an organ transplant
  2. Body weight <40 kg at screening
  3. Current plans for modification of the following background concomitant medications, as applicable, during screening and treatment period: erythropoietin, immunosuppressive drugs, corticosteroids, anti-thrombotic agents, anticoagulants, iron supplements, and folic acid as described in the protocol
  4. Any use of complement inhibitor therapy other than eculizumab in the 12 weeks prior to the screening visit or planned use during the study
  5. Known hypocellular bone marrow based on a history of reduced age-adjusted bone marrow cellularity and/or bone marrow cellularity ≤25%
  6. No documented meningococcal vaccination within 5 years prior to screening visit unless it is documented that vaccination has been administered during the screening period and prior to initiation of study treatment
  7. Unable to take antibiotics for meningococcal prophylaxis, if required by local standard of care
  8. Any active, ongoing infection or a recent infection requiring ongoing systemic treatment with antibiotics, antivirals, or antifungals within 2 weeks of screening or during the screening period
  9. Documented positive polymerase chain reaction (PCR) or equivalent test based on regional recommendations for COVID-19 or suspected SARS-CoV-2 infection as described in the protocol
  10. Documented history of active, uncontrolled, ongoing systemic autoimmune diseases
  11. Recent, unstable medical conditions, excluding PNH and PNH-related complications, within the past 3 months prior to screening visit as described in the protocol
  12. Anticipated need for major surgery during the study

NOTE: Other protocol-defined Inclusion/ Exclusion Criteria apply.

Study & Design

Study Type
INTERVENTIONAL
Study Design
SINGLE_GROUP
Arm && Interventions
GroupInterventionDescription
Pozelimab+CemdisiranCemdisiran-
Pozelimab+CemdisiranPozelimab-
Primary Outcome Measures
NameTimeMethod
Incidence and severity of treatment emergent adverse events (TEAEs)Through day 225

Open Label Treatment Period (OLTP)

Secondary Outcome Measures
NameTimeMethod
Change from baseline in concentration of total C5Up to Day 225

OLTP

Number of units of RBCs transfusedDay 1 through Week 52

OLEP

Proportion of participants who maintain adequate control of hemolysisDay 57 through Day 225

OLTP

Incidence and severity of TEAEs in participants treated with pozelimab and cemdisiran combination therapyThrough Week 52

Optional Open-Label Extension Period (OLEP)

Change of LDHDay 1 to Week 52

OLEP

Proportion of participants who maintain adequate control of their hemolysisDay 1 through Week 52

OLEP

Proportion of participants with normalization of LDH at each visitDay 1 through Week 52

OLEP

Change in fatigue as measured by FACIT-Fatigue scaleDay 1 to Week 52

OLEP

Concentrations of total pozelimab in serumUp to Week 52

OLEP

Concentrations of total C5Up to Week 52

OLEP

Concentrations of cemdisiran in plasmaUp to Week 52

OLEP

Percent change in LDH from pre-treatment to end-of-treatment periodScreening through Day 225

OLTP

Percent change in LDH from pre-treatmentScreening through Day 29

OLTP

Proportion of participants who are transfusion-freeDay 1 through Week 52

OLEP Not requiring an RBC transfusion as per protocol algorithm

Rate of RBCs transfusedDay 1 through Week 52

OLEP

Proportion of participants with breakthrough hemolysisDay 1 through Week 52

OLEP

Proportion of participants with adequate control of hemolysis at each visitDay 1 through Week 52

OLEP

Proportion of participants with normalization of their LDH at each visitPost Baseline (on Day 1) through Day 225

OLTP

Area under the curve (AUC) of LDH over timeBaseline through Day 225

OLTP

AUC of LDH over timeDay 1 through Week 52

OLEP

Proportion of participants with hemoglobin stabilizationDay 1 through Week 52

OLEP Participants who do not receive RBC transfusion and have no decrease in hemoglobin levels meeting pre-specified criteria.

Change in hemoglobin levelsDay 1 to Week 52

OLEP

Change in fatigue as measured by Functional Assessment of Chronic Illness Therapy-Fatigue (FACIT-Fatigue) scaleBaseline to Day 225

OLTP The FACIT-Fatigue is a 13-item, self-reported PRO measure assessing an individual's level of fatigue during their usual daily activities over the past week. This questionnaire is part of the FACIT measurement system, a compilation of questions measuring health-related QoL in patients with cancer and other chronic illnesses. The FACIT-fatigue assesses the level of fatigue using a Likert scale ranging from 0 (not at all) to 4 (very much). Scores range from 0 to 52, with higher scores indicating a higher quality of life.

Change in health related quality of life (HRQoL) as measured by the global health status subscale of the European Organization for Research and Treatment of Cancer (EORTC)- Quality of Life Cancer Patients Questionnaire (QLQ) - 30 ScaleBaseline to Day 225

OLTP EORTC-QLQ-C30 is a 30-item subject self-report questionnaire composed of both multi-item and single scales, including a global health status/quality of life (GHS/QoL) scale. Participants rate items on a four-point scale, with 1 as "not at all" and 4 as "very much." A change of 5 - 10 points is considered a small. A change of 10 - 20 points is considered a moderate change.

Change in physical function (PF) scores on the EORTC QLQ-C30Baseline to Day 225

OLTP

Change in total CH50Baseline to Day 225

OLTP

Concentrations of total pozelimab and eculizumab in serumUp to Day 225

OLTP

Concentrations of total cemdisiran in plasmaUp to Day 225

OLTP

Incidence of pozelimab anti-drug antibody (ADA) responses over timeUp to Week 52

OLEP

Incidence of cemdisiran anti-drug antibody (ADA) responses over timeUp to Week 52

OLEP

Incidence and severity of TEAEs for participants who receive dose intensificationThrough Day 225

Intensified OLTP

Percent change of LDHDay 1 to Week 52

OLEP

Change in GHS/QoL on the EORTC QLQ-C30Day 1 to Week 52

OLEP

Change in PF scores on the EORTC QLQ-C30Day 1 to Week 52

OLEP

Change in CH50Day 1 to Week 52

OLEP

Trial Locations

Locations (1)

Regeneron Study Site

🇬🇧

Leeds, United Kingdom

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