Canterbury DHB

Context

Acquired Haemophilia

Acquired Factor VIII Deficiency

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.

About this Canterbury DHB document (5270):

Document Owner:

Not assigned (see Who's Who)

Last Reviewed:

November 2019

Next Review:

November 2022

Keywords:

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Topic Code: 5270