Recombinant factor VIII Fc fusion protein: extended-interval dosing maintains low bleeding rates and correlates with von Willebrand factor levels

J Thromb Haemost. 2014 Nov;12(11):1788-800. doi: 10.1111/jth.12723. Epub 2014 Oct 10.

Abstract

Background: Routine prophylaxis with replacement factor VIII (FVIII) - the standard of care for severe hemophilia A - often requires frequent intravenous infusions (three or four times weekly). An FVIII molecule with an extended half-life could reduce infusion frequency. The A-LONG study established the safety, efficacy and prolonged pharmacokinetics of recombinant FVIII Fc fusion protein (rFVIIIFc) in previously treated adolescents and adults with severe hemophilia A.

Objective: In this post hoc analysis, we investigated the relationship between subjects' prestudy (FVIII) and on-study (rFVIIIFc) regimens.

Methods: We analyzed two subgroups of subjects: prior prophylaxis and on-study individualized prophylaxis (n = 80), and prior episodic treatment and on-study weekly prophylaxis (n = 16). Subjects' prestudy dosing regimens and bleeding rates were compared with their final rFVIIIFc regimens and annualized bleeding rates (ABRs) in the last 3 months on-study. Dosing regimen simulations based on population pharmacokinetics models for rFVIII and rFVIIIFc were performed.

Results: As compared with their prestudy regimen, 79 of 80 (98.8%) subjects on individualized rFVIIIFc prophylaxis decreased their infusion frequency. Overall ABRs were low, with comparable factor consumption. Longer dosing intervals, including 5-day dosing, were associated with higher baseline von Willebrand factor antigen levels. Simulated dosing regimens predicted a greater proportion of subjects with steady-state FVIII activity trough levels of ≥ 1 IU dL(-1) (1%) with rFVIIIFc than with equivalent rFVIII regimens.

Conclusion: These results suggest that patients on rFVIIIFc prophylaxis can reduce their infusion frequency as compared with their prior FVIII regimen while maintaining low bleeding rates, affording more patients trough levels of ≥ 1 IU dL(-1) than with rFVIII products requiring more frequent dosing regimens.

Keywords: administration schedule; drug; factor VIII; hemophilia A; pharmacokinetics; recombinant fusion proteins.

Publication types

  • Clinical Trial, Phase III
  • Comparative Study
  • Multicenter Study
  • Randomized Controlled Trial
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Biomarkers / blood
  • Coagulants / administration & dosage*
  • Coagulants / adverse effects
  • Coagulants / blood
  • Coagulants / pharmacokinetics
  • Computer Simulation
  • Drug Administration Schedule
  • Drug Monitoring
  • Factor VIII / administration & dosage*
  • Factor VIII / adverse effects
  • Factor VIII / pharmacokinetics
  • Half-Life
  • Hemophilia A / blood
  • Hemophilia A / diagnosis
  • Hemophilia A / drug therapy*
  • Hemorrhage / chemically induced
  • Hemorrhage / prevention & control*
  • Hemostasis / drug effects*
  • Humans
  • Immunoglobulin Fc Fragments / administration & dosage*
  • Immunoglobulin Fc Fragments / adverse effects
  • Immunoglobulin Fc Fragments / blood
  • Infusions, Intravenous
  • Male
  • Models, Biological
  • Recombinant Fusion Proteins / administration & dosage*
  • Recombinant Fusion Proteins / adverse effects
  • Recombinant Fusion Proteins / blood
  • Recombinant Fusion Proteins / pharmacokinetics
  • Severity of Illness Index
  • Time Factors
  • Treatment Outcome
  • Young Adult
  • von Willebrand Factor / metabolism*

Substances

  • Biomarkers
  • Coagulants
  • Immunoglobulin Fc Fragments
  • Recombinant Fusion Proteins
  • factor VIII-Fc fusion protein
  • von Willebrand Factor
  • Factor VIII