What is the primary treatment for hemophilia A with factor VIII (FVIII) inhibitors?

Updated: Jan 14, 2019
  • Author: Douglass A Drelich, MD; Chief Editor: Srikanth Nagalla, MBBS, MS, FACP  more...
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Answer

Inhibitors are antibodies that neutralize factor VIII (FVIII) and can render replacement therapy ineffective. They are found more commonly in patients with moderate to severe hemophilia (up to 30% of those with severe disease) who have received significant amounts of replacement therapy. Inhibitors develop in relatively young children, usually within their first 50 exposures to FVIII.

Rarely, inhibitors can develop in individuals without genetic hemophilia (eg, elderly persons, pregnant women). These occasionally are responsive to immunosuppressive therapy (eg, prednisone).

The treatment of patients with inhibitors of FVIII is difficult. Assuming no anamnestic response, low-titer inhibitors (ie, concentrations below 5 Bethesda units [BU]) occasionally can be overcome with high doses of factor VIII. [29] There is no established treatment for bleeding episodes in patients with high-titer inhibitors.


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