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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
OLTP: Number of Participants With Treatment-emergent Adverse Events (TEAEs)Up to Day 225
Secondary Outcome Measures
NameTimeMethod
OLEP: Number of Participants With Normalization of Their LDH at Each Visit in OLEPBaseline, Days 57, 113, 169, 225, 281, 365 of the OLEP

Normalization was defined as LDH ≤ 1.0 x ULN at that visit.

OLTP: Percent Change in Lactate Dehydrogenase (LDH) From Pre-treatment to End-of-treatment PeriodScreening (Day 1) through Day 225
OLTP: Percent Change in LDH From Pre-treatment Through Day 29Screening (Day 1) through Day 29
OLTP: Percentage of Participants Who Were Transfusion-free From Baseline Through Week 32Baseline through Week 32
OLTP: Percentage of Participants Who Were Transfusion-free From Week 4 Through Week 32Week 4 through Week 32
OLTP: Rate of Red Blood Cell (RBC) Transfusions From Baseline Through Week 32Baseline through Week 32

The rate of units of transfusion for a participant was calculated based on the duration of treatment exposure of the participant.

OLTP: Rate of RBC Transfusions From Week 4 Through Week 32Week 4 through Week 32

The rate of units of transfusion for a participant was calculated based on the duration of treatment exposure of the participant.

OLTP: Number of RBC Units Transfused From Baseline Through Week 32Baseline through Week 32
OLTP: Number of RBC Units Transfused From Week 4 Through Week 32Week 4 through Week 32
OLTP: Percentage of Participants With Breakthrough Hemolysis From Baseline Through Week 32Baseline through Week 32

Breakthrough hemolysis was defined as having an LDH ≥ 2 x upper limit of normal (ULN) and having signs or symptoms.

OLTP: Percentage of Participants With Breakthrough Hemolysis From Week 4 Through Week 32Week 4 through Week 32

Breakthrough hemolysis was defined as having an LDH ≥ 2 x ULN and having signs or symptoms.

OLTP: Percentage of Participants Who Maintained Adequate Control of Hemolysis From Day 1 Through Week 32Day 1 through Week 32

Adequate control was defined as LDH ≤ 1.5 x ULN from Day 1 through Week 32.

OLTP: Percentage of Participants Who Maintained Adequate Control of Hemolysis From Week 8 Through Week 32Week 8 through Week 32

Adequate control was defined as LDH ≤ 1.5 x ULN from Week 8 through Week 32.

OLTP: Number of Participants With Adequate Control of Hemolysis at Each VisitDays 1, 8, 15, 29, 43, 57, 71, 85, 113, 141, 169, 197, 225

Adequate control at a visit was defined as having LDH ≤1.5 x ULN at that visit.

OLTP: Number of Participants With Normalization of Their LDH at Each VisitDays 1, 8, 15, 29, 43, 57, 71, 85, 113, 141, 169, 197, 225

Normalization was defined as LDH ≤ 1.0 x ULN at that visit.

OLTP: Average LDH From Baseline Through Week 32Baseline through Week 32

The trapezoidal rule was used to calculate area under the curve (AUC). Individual mean LDH is defined as AUC divided by (last assessment date of LDH - first assessment date of LDH) of specific period.

OLTP: Average LDH From Week 8 Through Week 32Week 8 through Week 32

The trapezoidal rule was used to calculate AUC. Individual mean LDH is defined as AUC divided by (last assessment date of LDH - first assessment date of LDH) of specific period.

OLTP: Percentage of Participants With Hemoglobin Stabilization From Baseline Through Week 32Baseline through Week 32

Hemoglobin stabilization was defined as not receiving an RBC transfusion and having no decrease in hemoglobin level of ≥ 2 grams per deciliter (g/dL).

OLTP: Percentage of Participants With Hemoglobin Stabilization From Week 4 Through Week 32Week 4 through Week 32

Hemoglobin stabilization was defined as not receiving an RBC transfusion and having no decrease in hemoglobin level of ≥ 2 g/dL.

OLTP: Change From Baseline in Hemoglobin LevelsBaseline, Week 32
OLEP: Average LDH From Day 1 Through Week 24 of the OLEPDay 1 through Week 24 of the OLEP

The trapezoidal rule was used to calculate AUC. Individual mean LDH is defined as AUC divided by (last assessment date of LDH - first assessment date of LDH) of specific period.

OLTP: Change From Baseline in Fatigue as Measured by Functional Assessment of Chronic Illness Therapy-Fatigue (FACIT-Fatigue) ScoreBaseline, Week 32

The FACIT-Fatigue is a 13-item, self-reported 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 quality of life (QoL) in participants 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). Total scores range from 0 to 52, with higher scores indicating a higher quality of life.

OLTP: Change From Baseline 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, Week 32

EORTC-QLQ-C30 is a 30-item self-reported questionnaire composed of both multi-item and single scales, including a global health status/quality of life (GHS/QoL) scale. The GHS subscale scores ranged from 0 to 100 with higher scores indicating better quality of life.

OLTP: Change From Baseline in Physical Function (PF) Scores on the EORTC QLQ-C30Baseline, Week 32

EORTC-QLQ-C30 is a 30-item participant self-report questionnaire composed of both multi-item and single scales, including a PF scale. PF subscale scores ranged from 0 to 100 with higher scores indicating better quality of life.

OLTP: Change From Baseline in Total Complement Hemolytic Activity Assay (CH50)Baseline through Week 32

This assay assessed the activity of the classical pathway of complement to measure C5 activity.

OLTP and OLEP: Concentration of Total Pozelimab in SerumPre-dose Days 28, 56, 84, 112, 140, 168, 196, 224, 392, 588; Post-dose Day 28
OLTP: Concentration of Total Eculizumab in SerumPre-dose Days 0, 14, 28, 56, 84, 112, 140, 168, 196, 224
OLTP and OLEP: Concentration of Total Cemdisiran in PlasmaPre-dose and Post-dose Days 0, 84, 196, 224, 588
OLTP and OLEP: Concentration of Total C5 in PlasmaPre-dose Days 0, 7, 14, 28, 56, 84, 112, 140, 168, 196, 224, 392, 588
OLTP: Number of Participants With Pozelimab Anti-drug Antibodies (ADA)Baseline through Week 32
OLTP: Number of Participants With Cemdisiran ADABaseline through Week 32
OLTP: Number of Participants Who Received Dose Intensification With TEAEsBaseline through Week 32
OLEP: Number of Participants With TEAEsDay 1 through Week 52 of the OLEP
OLEP: Percent Change in LDH From Day 1 Through Week 24 of the OLEPDay 1 through Week 24 of the OLEP
OLEP: Percent Change in LDH From Day 1 Through Week 52 of the OLEPDay 1 through Week 52 of the OLEP
OLEP: Percentage of Participants Who Were Transfusion-free From Day 1 Through Week 24 of the OLEPDay 1 through Week 24 of the OLEP
OLEP: Percentage of Participants Who Were Transfusion-free From Day 1 Through Week 52 of the OLEPDay 1 through Week 52 of the OLEP
OLEP: Rate of RBC Transfusions From Day 1 Through Week 24 of the OLEPDay 1 through Week 24 of the OLEP

The rate of units of transfusion for a participants was calculated based on the duration of treatment exposure of the participant.

OLEP: Rate of RBC Transfusions From Day 1 Through Week 52 of the OLEPDay 1 through Week 52 of the OLEP

The rate of units of transfusion for a participants was calculated based on the duration of treatment exposure of the participant.

OLEP: Number of RBC Units Transfused From Day 1 Through Week 24 of the OLEPDay 1 through Week 24 of the OLEP
OLEP: Number of RBC Units Transfused From Day 1 Through Week 52 of the OLEPDay 1 through Week 52 of the OLEP
OLEP: Percentage of Participants With Breakthrough Hemolysis From Day 1 Through Week 24 of the OLEPDay 1 through Week 24 of the OLEP

Breakthrough hemolysis was defined as having an LDH ≥ 2 x ULN and having signs or symptoms.

OLEP: Percentage of Participants With Breakthrough Hemolysis From Day 1 Through Week 52 of the OLEPDay 1 through Week 52 of the OLEP

Breakthrough hemolysis was defined as having an LDH ≥ 2 x ULN and having signs or symptoms.

OLEP: Change From Baseline in HRQoL as Measured by the Global Health Status Subscale of the EORTC-QLQ-30 ScaleDay 1 and Week 52 of the OLEP

EORTC-QLQ-C30 is a 30-item self-reported questionnaire composed of both multi-item and single scales, including a global health status/quality of life (GHS/QoL) scale. GHS subscale scores ranged from 0 to 100 with higher scores indicating better quality of life.

OLEP: Change From Baseline in PF Scores on the EORTC QLQ-C30Day 1 and Week 52 of the OLEP

EORTC-QLQ-C30 is a 30-item participant self-report questionnaire composed of both multi-item and single scales, including a PF scale. PF subscale scores ranged from 0 to 100 with higher scores indicating better quality of life.

OLEP: Change From Baseline in CH50Baseline, Week 16, Week 32, Week 52 of the OLEP

This assay assessed the activity of the classical pathway of complement to measure C5 activity.

OLEP: Number of Participants With Pozelimab ADADay 1 through Week 52 of the OLEP
OLEP: Number of Participants With Cemdisiran ADADay 1 through Week 52 of the OLEP
OLEP: Percentage of Participants Who Maintained Adequate Control of Hemolysis From Day 1 Through Week 24 of the OLEPDay 1 through Week 24 of the OLEP

Adequate control was defined as LDH ≤ 1.5 x ULN.

OLEP: Percentage of Participants Who Maintained Adequate Control of Hemolysis From Day 1 Through Week 52 of the OLEPDay 1 through Week 52 of the OLEP

Adequate control was defined as LDH ≤ 1.5 x ULN.

OLEP: Number of Participants Who Maintained Adequate Control of Hemolysis at Each Visit in OLEPBaseline, Days 57, 113, 169, 225, 281, 365 of the OLEP

Adequate control was defined as LDH ≤ 1.5 x ULN at that visit.

OLEP: Average LDH From Day 1 Through Week 52 of the OLEPDay 1 through Week 52 of the OLEP

The trapezoidal rule was used to calculate AUC. Individual mean LDH is defined as AUC divided by (last assessment date of LDH - first assessment date of LDH) of specific period.

OLEP: Percentage of Participants With Hemoglobin Stabilization From Day 1 Through Week 24 of the OLEPDay 1 through Week 24 of the OLEP

Hemoglobin stabilization was defined as not receiving an RBC transfusion and having no decrease in hemoglobin level of ≥ 2 g/dL.

OLEP: Percentage of Participants With Hemoglobin Stabilization From Day 1 Through Week 52 of the OLEPDay 1 through Week 52 of the OLEP

Hemoglobin stabilization was defined as not receiving an RBC transfusion and having no decrease in hemoglobin level of ≥ 2 g/dL.

OLEP: Change From Baseline in Hemoglobin LevelsDay 1 and Week 52 of the OLEP
OLEP: Change From Baseline in FACIT-Fatigue ScoreDay 1 and Week 52 of the OLEP

The FACIT-Fatigue is a 13-item, self-reported 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 quality of life (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). Total scores range from 0 to 52, with higher scores indicating a higher quality of life.

Trial Locations

Locations (1)

Regeneron Study Site

🇬🇧

Leeds, United Kingdom

Regeneron Study Site
🇬🇧Leeds, United Kingdom
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