Acquired haemophilia A is a rare auto-immune disease caused by an inhibitory antibody to factor VIII. Patients with this disorder are at high risk of severe bleeding until the inhibitor has been eradicated. Management of this disorder consists in rapid accurate diagnosis, control of bleeding and eradication of the inhibitor by immunosuppression. The cessation of bleeding is based mainly on recombinant factor VIIa and activated prothrombin complex concentrate which are approximately equally efficacious. Immunosuppression is still based on steroids alone or with combination with cyclopfosphamide which may result in a higher rate of remission. New drugs as rituximab or cyclosporine A are the second line option. In case of life-threatening bleeding immunoadsorption and high dose of fac-tor VIII could be advantageous.

Oddeleni klinicke hematologie FN Brno