Diagnosis and treatment of acquired haemophilia

F Baudo1, T Caimi, F de Cataldo

  • 1Thrombosis and Hemostasi Unit, Niguarda Hospital, Milan, Italy. francesco.baudo@ospedaleniguarda.it

Acquired haemophilia (AH) is an autoimmune syndrome characterized by acute bleeding in patients with negative family and personal history, and factor VIII depletion. Its incidence is 1.6 x 106 population per year. AH is associated with autoimmune diseases, solid tumours, lymphoprolipherative diseases, pregnancy; 50% of the cases idiopathic. Spontaneous or after minor trauma severe bleeding associated with a prolonged activated partial thromboplastin time, not corrected by incubation with normal plasma, with a normal prothrombin time are the diagnostic hallmarks. The goals of management are the control of bleeding and the suppression of inhibitor. First-line haemostatic treatment includes recombinant factor VIIa and activated prothrombin complex concentrate. Prednisone +/- cyclophosphamide and other immunosuppressive agents are the standard intervention for inhibitor eradication.

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