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Phase 3 N=86 Randomized Double-blind Treatment

Danicopan as Add-on Therapy to a C5 Inhibitor in Paroxysmal Nocturnal Hemoglobinuria (PNH) Participants Who Have Clinically Evident Extravascular Hemolysis (EVH)(ALPHA)

Paroxysmal Nocturnal Hemoglobinuria

Enrolled (actual)
86
Serious AEs
10.9%
Results posted
Jul 2023
Primary outcome: Primary: Change From Baseline in Hgb at Week 12 — 29.40; 4.96; 28.08; 4.62 g/L — p=0.0007

Study Design & Population

Study type
Interventional
Phase
Phase 3
Interventions
Danicopan (Drug); Placebo (Drug); C5 Inhibitor (Drug)
Age
Adult, Older Adult · 18+ yrs
Sex
All
Sponsor
Alexion Pharmaceuticals, Inc.
Primary completion
Jun 2022

Outcome Measures

OutcomeResultp-value
PRIMARY
Change From Baseline in Hgb at Week 12
29.40; 4.96; 28.08; 4.62 0.0007 sig
SECONDARY
Percentage of Participants With Hgb Increase of ≥2 Grams/Deciliter (g/dL) (≥20 g/L) From Baseline in the Absence of Transfusion at Week 12
59.5; 0; 54.4; 0
SECONDARY
Percentage of Participants With Transfusion Avoidance Through Week 12
83.3; 38.1; 78.9; 27.6
SECONDARY
Change From Baseline in Functional Assessment of Chronic Illness Therapy (FACIT) Fatigue Score at Week 12
7.97; 1.85; 8.13; 2.35
SECONDARY
Change From Baseline in Absolute Reticulocyte Count at Week 12
-0.0838; 0.0035; -0.0925; -0.0008
SECONDARY
Change in the Number of Red Blood Cell (RBC) Units Transfused From 24 Weeks Prior to Initiation of Treatment to Post 24 Weeks of Treatment
-2.7
SECONDARY
Change in Number of Transfusion Instances From 24 Weeks Prior to Initiation of Treatment to Post 24 Weeks of Treatment
-1.5
SECONDARY
Percentage of Participants With Transfusion Avoidance Through Week 24
69.1
SECONDARY
Change in the Number of RBC Units Transfused From 12 Weeks Prior to Initiation of Treatment to Post 12 Weeks of Treatment
-1.48; -0.18; -1.44; -0.14
SECONDARY
Change in Number of Transfusion Instances From 12 Weeks Prior to Initiation of Treatment to Post 12 Weeks of Treatment
-0.92; -0.21; -0.91; -0.11
SECONDARY
Change From Baseline FACIT Fatigue Scores at Week 24
6.21; 5.64
SECONDARY
Percentage of Participants With Hgb Stabilization During Last 12 Weeks of Treatment in Participants Receiving 24 Weeks of Danicopan
58.2
SECONDARY
Percentage of Participants With Hgb Increase of ≥2 g/dL (≥ 20 g/L) From Baseline in the Absence of Transfusion at Week 24
41.8
SECONDARY
Change From Baseline in Total and Direct Bilirubin at Week 12
-9.77; -2.15; -2.88; 0.30; -11.55; -1.42
SECONDARY
Change From Baseline in Paroxysmal Nocturnal Hemoglobinuria (PNH) RBC Clone Size at Week 12
24.60; -3.04; 26.35; -0.18
SECONDARY
Change From Baseline in Complement Component 3 Fragment Deposition (C3d PNH Type 3 Cells) on PNH RBCs at Week 12
-15.06; 0.89; -19.00; 0.68
SECONDARY
Change From Baseline in Lactate Dehydrogenase at Week 12
-23.49; -2.92; -25.60; -16.92
SECONDARY
Percentage of Participants With Hgb Normalization at Week 12
28.6; 0; 26.3; 0
SECONDARY
Percentage of Participants With Hgb Normalization at Week 24
20.0

Summary

The main objective of this study is to evaluate the efficacy of danicopan as add-on therapy to a complement component 5 (C5) inhibitor (eculizumab or ravulizumab) in participants with PNH who have clinically evident EVH.

Eligibility Criteria

Inclusion Criteria

  • Diagnosis of PNH
  • Clinically Evident EVH defined by:
  • Anemia (Hgb ≤9.5 gram/deciliter) with absolute reticulocyte count ≥120 x 10^9/liter
  • Receiving an approved C5 inhibitor for at least 6 months prior to Day 1
  • Platelet count ≥30,000/microliters (µL)
  • Absolute neutrophil counts ≥500/μL
  • Documentation of/or willingness to receive vaccinations for N. meningiditis and prophylactic antibiotics as required

Exclusion Criteria

  • History of a major organ transplant or hematopoietic stem cell transplantation (HSCT)
  • Participants with known aplastic anemia or other bone marrow failure that requires HSCT or other therapies including anti-thymocyte globulin and/or immunosuppressants
  • Known or suspected complement deficiency
  • Laboratory abnormalities at screening, including:
  • Alanine aminotransferase >2 x ULN (>3 x ULN in case of patients with documented liver iron overload defined by serum ferratin values
  • 500 ng/ML)
  • Direct bilirubin >2 x ULN (unless due to EVH or documented Gilbert's Syndrome)
  • Current evidence of biliary cholestasis
  • Estimated glomerular filtration rate of <30 milliliters/minute/1.73 meter squared and/or are on dialysis
  • Evidence of human immunodeficiency virus, hepatitis B, or active hepatitis C infection at screening
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT04469465). Outcome figures and adverse-event rates are extracted automatically from the registry's posted results and are provided for clinician reference, not as a substitute for the primary publication.

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