Hemophilia A (Factor VIII Deficiency) Guidelines

Updated: Jul 05, 2023
  • Author: Douglass A Drelich, MD; Chief Editor: Srikanth Nagalla, MD, MS, FACP  more...
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Guidelines Summary

Guidelines on the management of acute joint bleeds and chronic synovitis in hemophilia were issued by the United Kingdom Haemophilia Centre Doctors' Organisation in 2017. [74] Recommendations on hemostatic management of patients with hemophilia A without inhibitors were as follows:

  • All patients with severe hemophilia A and other patients at risk of joint bleeding should be offered home treatment.
  • The initial treatment of early and moderate bleeds should aim for a peak factor VIII (FVIII) of 50 to 60 IU dL −1. This is equivalent to 25 to 30 IU kg −1 for severe hemophilia A for standard and extended half-life products. 
  • Early bleeds often do not require a second infusion, and moderate bleeds often respond to a single infusion but may require up to 2 infusions.
  • Children may require more frequent or higher doses, as they have a shorter factor half-life than adults.
  • For joint-immobilizing bleeds, higher initial doses are recommended, which aim to raise the peak FVIII level to 60 to 80 IU dL −1. Doses should be administered every 24 hr until complete resolution of pain. For severe bleeds, more frequent administration may be required in the initial 48 hr with standard FVIII.

Recommendations for hemostatic management of patients with inhibitors to FVIII were as follows:

  • Inhibitor patients should be encouraged to be on a home treatment program, and bleeds should be treated as early as possible.
  • Activated prothrombin complex (aPCC) 50–100 μg kg −1 or recombinant factor VIIa (rFVIIa) 270 μg kg −1 as a single dose (or 90 μg kg −1 2–3 hourly) are equally acceptable treatments for joint or soft tissue bleeds with repeated doses as necessary. The frequency of infusion and duration of treatment should be determined by the clinical response.
  • The total daily dose of aPCC should not exceed 200 IU kg −1.
  • Tranexamic acid can be considered as adjunctive therapy to aPCC and rFVIIa.
  • Sequential alternating treatment of aPCC and rFVIIa can be considered for the management of limb/life-threatening bleeds.