Canterbury DHB
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Individuals who are not haemophiliac may develop autoantibodies directed at Factor VIII. In most cases, no underlying disease can be found, but these autoantibodies may be seen post partum, with autoimmune disease, lymphoid malignancies, or in association with drugs.
This is a rare condition with a high mortality rate of around 20%. This may be reduced with prompt diagnosis and treatment.
Treatment of bleeding consists of inhibitor by-passing therapy. The Factor VIII concentrates normally used for haemophilia treatment are usually ineffective. FEIBA and/or Novo Seven® may be required to control bleeding.
These inhibitors may gradually disappear but immunosuppressive treatment should be started as soon as the diagnosis is made. Corticosteroids, e.g., 1-2 mg/kg/day, cytotoxic agents, especially cyclophosphamide, e.g., 2-3 mg/kg/day PO, and IV Immunoglobulin, 1-2 g over 1 or 2 days, may all lead to improvement. There are reports of Rituximab being effective in this condition.
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Topic Code: 5270
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