Management of patients with spontaneous inhibitor

• Severe bleeding—may be life threatening. Suppress inhibitor with prednisolone (1mg/kg/d); may take weeks to work, cyclophosphamide can be added. Treat active bleeding. If no cross-reactivity porcine VIII concentrates can be used if available (50—150u/kg). FEIBA and recombinant VIIa are also effective as 'bypassing agents'.

• Mild bleeding—may respond local pressure, tranexamic acid or DDAVP (M p354).

• Monitor lab and clinical response.

• Long term immunosuppression may be required.

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