Diagnosis and treatment of acquired haemophilia.
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.
Thrombosis and Hemostasi Unit, Niguarda Hospital, Milan, Italy. francesco.baudo@ospedaleniguarda.it
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D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D019774:Blood Coagulation Factor Inhibitors / Q000032:analysis; D001779:Blood Coagulation Factors / Q000008:administration & dosage / Q000627:therapeutic use*; D005169:Factor VIII / Q000008:administration & dosage / Q000627:therapeutic use*; D015942:Factor VIIa / Q000008:administration & dosage / Q000627:therapeutic use*; D005260:Female; D006467:Hemophilia A / Q000175:diagnosis* / Q000628:therapy*; D006470:Hemorrhage / Q000517:prevention & control; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D010314:Partial Thromboplastin Time; D011994:Recombinant Proteins / Q000008:administration & dosage / Q000627:therapeutic use
D019774:Blood Coagulation Factor Inhibitors; D001779:Blood Coagulation Factors; D007166:Immunosuppressive Agents; D011994:Recombinant Proteins; C025667:prothrombin complex concentrates; D005169:Factor VIII; C103587:recombinant FVIIa; D015942:Factor VIIa