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Phase 3 N=40 Treatment

Study of Efficacy and Safety of Twice Daily Oral Iptacopan (LNP023) in Adult PNH Patients Who Are Naive to Complement Inhibitor Therapy

Paroxysmal Nocturnal Hemoglobinuria (PNH)

Enrolled (actual)
40
Serious AEs
20.0%
Results posted
Nov 2023
Primary outcome: Primary: Marginal Proportion (Expressed as Percentage) of Participants With Sustained Increase in Hemoglobin Levels From Baseline of ≥ 2 g/dL in the Absence of Red Blood Cell Transfusions — 92.2 Percentage of responders

Study Design & Population

Study type
Interventional
Phase
Phase 3
Interventions
Iptacopan (LNP023) (Drug)
Age
Adult, Older Adult · 18+ yrs
Sex
All
Sponsor
Novartis Pharmaceuticals
Primary completion
Nov 2022

Outcome Measures

OutcomeResultp-value
PRIMARY
Marginal Proportion (Expressed as Percentage) of Participants With Sustained Increase in Hemoglobin Levels From Baseline of ≥ 2 g/dL in the Absence of Red Blood Cell Transfusions
92.2
SECONDARY
Marginal Proportion (Expressed as Percentage) With Sustained Hemoglobin Levels of ≥ 12 g/dL in the Absence of Red Blood Cell Transfusions
62.8
SECONDARY
Marginal Proportion (Expressed as Percentage) of Participants Who Remain Free From Transfusions
97.6
SECONDARY
Change From Baseline in Hemoglobin Levels in the Core Treatment Period
4.28
SECONDARY
Percent Change From Baseline in LDH
-83.55
SECONDARY
Adjusted Annualized Clinical BTH Rate in the Core Treatment Period
0.00
SECONDARY
Change From Baseline in Absolute Reticulocyte Count
-82.48
SECONDARY
Change From Baseline in FACIT-Fatigue Score
10.4
SECONDARY
Adjusted Annualized Major Adverse Vascular Events Rate in the Core Treatment Period
0.00

Summary

The purpose of this Phase 3 study was to determine whether iptacopan is efficacious and safe for the treatment of Paroxysmal nocturnal hemoglobinuria (PNH) patients who were naïve to complement inhibitor therapy.

Eligibility Criteria

Inclusion Criteria

  • Male and female participants ≥ 18 years of age with a diagnosis of PNH confirmed by high-sensitivity flow cytometry with RBCs and WBCs clone size ≥ 10%
  • Mean hemoglobin level 1.5 x Upper Limit of Normal (ULN)
  • Vaccination against Neisseria meningitidis infection is required prior to the start of study treatment
  • If not received previously, vaccination against Streptococcus pneumoniae and Haemophilus influenzae infections should be given

Exclusion Criteria

  • Prior treatment with a complement inhibitor, including anti-C5 antibody
  • Known or suspected hereditary complement deficiency
  • History of hematopoietic stem cell transplantation
  • Patients with laboratory evidence of bone marrow failure (reticulocytes <100x109/L; platelets <30x109/L; neutrophils <0.5x109/L).
  • Active systemic bacterial, viral (incl. COVID-19)or fungal infection within 14 days prior to study drug administration.
  • History of recurrent invasive infections caused by encapsulated organisms, e.g. meningococcus or pneumococcus.
  • Major concurrent comorbidities including but not limited to severe kidney disease (e.g., dialysis), advanced cardiac disease (e.g., NYHA class IV heart failure), severe pulmonary disease (e.g., severe pulmonary hypertension (WHO class IV)), or hepatic disease (e.g., active hepatitis) that in the opinion of the investigator precludes participant's participation in the study.
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT04820530). 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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