Life-threatening Bleeding in Hemophilia A Patients

• Although an infrequent occurrence, patients with Hemophilia A can present with life-threatening hemorrhage (e.g. ICH).
• Recall that normal clotting factor levels range from 50-150 IU/dL - reported by the lab as 50-150%.
• Life-threatening bleeding requires Factor VIII levels between 80-100%.  In general, each unit of FVIII/kg raises plasma levels by 2%.
• Recombinant Factor VIII products are preferred over plasma derived concentrates or blood products and are dosed as:
  • FVIII - 50 IU/kg loading dose followed by infusion of 3 IU/kg/hr
• In the event you don't have access to recombinant or plasma derived FVIII concentrates, cryoprecipitate (contains FVIII) can be used.

References

Singleton T, Kruse-Jarres R, Leissinger C. Emergency department care for patients with Hemophilia and Von Willebrand Disease. JEM 2010; 39:158-65.