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 start immunosuppressive treatment as soon as the diagnosis is made. Corticosteroids, e.g. 1 to 2 mg/kg/day, cytotoxic agents, especially cyclophosphamide, e.g. 2 to 3 mg/kg/day PO, and IV Immunoglobulin, 1 to 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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