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Phase 3 N=1 Treatment

Single-Arm Study To Evaluate The Efficacy and Safety of Valoctocogene Roxaparvovec in Hemophilia A Patients at a Dose of 4E13 vg/kg

Hemophilia A

Enrolled (actual)
1
Serious AEs
0.0%
Results posted
Oct 2021
Primary outcome: Primary: Change of the Median Factor VIII (FVIII) Activity — 4.1 IU/dL

Study Design & Population

Study type
Interventional
Phase
Phase 3
Interventions
Valoctocogene Roxaparvovec (Biological)
Age
Adult, Older Adult · 18+ yrs
Sex
Male
Sponsor
BioMarin Pharmaceutical
Primary completion
May 2019

Outcome Measures

OutcomeResultp-value
PRIMARY
Change of the Median Factor VIII (FVIII) Activity
4.1
SECONDARY
Change in the Annualized Utilization (IU/kg) of Exogenous FVIII Replacement Therapy
-4058.24
SECONDARY
Change in the Annualized Number of Bleeding Episodes Requiring Exogenous FVIII Replacement Treatment
5.77

Summary

This Phase III clinical study will assess the efficacy of BMN 270 defined as FVIII activity, during weeks 49-52 following intravenous infusion of BMN 270 and assess the impact of BMN 270 on usage of exogenous FVIII replacement therapy and the number of bleeding episodes from week 5 to week 52.

Eligibility Criteria

Inclusion Criteria

  • Males ≥ 18 years of age with hemophilia A and residual FVIII levels ≤ 1 IU/dL as evidenced by medical history.
  • Must have been on prophylactic FVIII replacement therapy for at least 12 months prior to study entry.
  • Treated/exposed to FVIII concentrates or cryoprecipitate for a minimum of 150 exposure days.
  • No previous documented history of a detectable FVIII inhibitor of less than 0.6 Bethesda Units (BU).

Exclusion Criteria

  • Detectable pre-existing antibodies to the AAV5 capsid.
  • Any evidence of active infection or any immunosuppressive disorder, including HIV infection.
  • Significant liver dysfunction, prior liver biopsy showing significant fibrosis, liver cirrhosis of any etiology or history of hepatic malignancy.
  • Evidence of any bleeding disorder not related to hemophilia A.
  • Active Hepatitis C.
  • Prior treatment with any vector/gene transfer agent.
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT03392974). 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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