Diagnosis and treatment of acquired haemophilia

Authors


Francesco Baudo, Thrombosis Hemostasis Unit, Niguarda Hospital, Milano, Italy.
Tel.: +39 335 225 606; fax: +39 02 6444 3948;
e-mail francesco.baudo@ospedaleniguarda.it

Abstract

Summary.  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 × 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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