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Phase 3 N=67 Treatment

HOPE-B: Trial of AMT-061 in Severe or Moderately Severe Hemophilia B Patients

Hemophilia B

Enrolled (actual)
67
Serious AEs
16.6%
Results posted
Oct 2022
Primary outcome: Primary: Annualized Bleeding Rate (ABR) for All Bleeding Episodes — 4.19; 1.51 bleeds/year/participants

Study Design & Population

Study type
Interventional
Phase
Phase 3
Interventions
AAV5-hFIXco-Padua (Genetic); Factor IX (FIX) (Biological)
Age
Adult, Older Adult · 18+ yrs
Sex
Male
Sponsor
CSL Behring
Primary completion
Sep 2021

Outcome Measures

OutcomeResultp-value
PRIMARY
Annualized Bleeding Rate (ABR) for All Bleeding Episodes
4.19; 1.51
SECONDARY
Factor IX Activity Levels After AMT-061 (CSL222) Dosing
1.19; 38.95; 41.48; 36.90
SECONDARY
Annualized Exogenous FIX Consumption
257,338.8; 12,912.9; 8399.1; 8486.6
SECONDARY
Adjusted Annualized Infusion Rate of FIX Replacement Therapy
72.49; 2.53
SECONDARY
Percent of Participants Who Discontinued FIX Prophylaxis and Remained Free of Routine FIX Prophylaxis After AMT-061 (CSL222) Dosing
96.3
SECONDARY
Percentage of Participants With Trough FIX Activity <12% of Normal
79.6; 7.8; 8.0; 6.0
SECONDARY
ABR for FIX-treated Bleeding Episodes
3.65; 0.84
SECONDARY
Annualized Rate of Spontaneous Bleeding Episodes
1.52; 0.44
SECONDARY
Annualized Rate of Joint Bleeding Episodes
2.35; 0.51
SECONDARY
Mean FIX Activity (%) in Participants With Pre-Existing Neutralizing Antibodies to AAV5 After AMT-061 (CSL222) Dosing
1.24; 35.91; 35.54; 31.14
SECONDARY
Mean FIX Activity (%) in Participants Without Pre-Existing Neutralizing Antibodies to AAV5 After AMT-061 (CSL222) Dosing
1.15; 40.61; 44.82; 39.87
SECONDARY
Number of New Target Joints and the Number of New Target Joints Resolved
1; 0
SECONDARY
Percent of Participants With Zero Bleeding Episodes During the 52 Weeks Following Stable FIX Expression (6 to 18 Months) After AMT-061 (CSL222) Dosing
25.9; 63.0
SECONDARY
International Physical Activity Questionnaire (iPAQ) Overall Score
4548.1; 3826.9
SECONDARY
EuroQol-5 Dimensions-5 Levels (EQ-5D-5L) VAS Overall Score
80.9; 81.0
SECONDARY
Number of Participants With Adverse Events
54
SECONDARY
Number of Participants With Change (Shift) in Abdominal Ultrasound Results From Normal, Abnormal and Missing to Abnormal Post Treatment
21; 22; 1
SECONDARY
Number of Participants With Anti-AAV5 Antibodies
49; 8; 50
SECONDARY
Number of Participants With AAV5 Capsid-specific T Cells
5
SECONDARY
Number of Participants With Anti-FIX Antibodies
48; 1
SECONDARY
Number of Participants With FIX Inhibitors and Recovery
SECONDARY
Number of Participants With Increased Aspartate Aminotransferase (AST) and Alanine Aminotransferase (ALT) Levels And Who Used Any Corticosteroids For Treatments
13; 10; 9
SECONDARY
Number of Participants With Newly Occurring or Worsening Potentially Clinically Significant Laboratory Values
1; 1; 11; 19; 2
SECONDARY
Number of Participants With Vector DNA Shedding
45; 49
SECONDARY
Number of Participants With Inflammatory Markers
45; 1; 0; 17; 53
SECONDARY
Number of Participants With Alpha-fetoprotein (AFP) Levels Above LLOQ at Month 60
43

Summary

This is an open-label, single-dose, multi-center, multinational trial to demonstrate the efficacy of AMT-061 and to further describe its safety profile. The study drug is identified as AAV5-hFIXco-Padua (AMT- 061). AMT-061 is a recombinant adeno-associated viral vector of serotype 5 (AAV5) containing the Padua variant of a codon-optimized human FIX complementary deoxyribonucleic acid (cDNA) under the control of a liver-specific promoter. The pharmaceutical form of AMT-061 is a solution for intravenous infusion administered at a dose of 2 x 10^13 gc/kg.

Eligibility Criteria

Inclusion Criteria

  • Male
  • Age ≥18 years
  • Subjects with congenital hemophilia B, classified as severe or moderately severe, and are currently on factor IX prophylaxis
  • >150 previous exposure days of treatment with factor IX protein

Exclusion Criteria

  • History of factor IX inhibitors
  • Positive factor IX inhibitor test at screening
  • Select screening laboratory value >2 times upper limit of normal
  • Positive human immunodeficiency virus (HIV) test at screening, not controlled with anti-viral therapy
  • Active infection with hepatitis B or C virus at screening
  • History of Hepatitis B or C exposure, currently controlled by antiviral therapy at the end of the lead-in phase
  • Previous gene therapy treatment
  • Receipt of an experimental agent within 60 days prior to screening
  • Current participation or anticipated participation within one year after study drug administration in this trial in any other interventional clinical trial involving drugs or devices
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT03569891). 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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