ACQUIRED HEMOPHILIA

Acquired hemophilia is a very rare disease characterized by the presen ce of an autoantibody (mainly IgG) to factor VIII, with a clinical pre sentation resembling hemophilia A. It is associated with various autoi mmune or dermatologic diseases, pregnancy, cancer, or drug ingestion, but in almost 50% of patients no underlying disorder is found. The tre atment of acquired hemophilia is particularly complex because response to therapy is unpredictable. If an acute hemorrhage occurs despite pr eventive measures, two complementary strategies must be employed: stop ping the bleeding and decreasing the factor VIII inhibitor with human or porcine factor VIII, DDAVP, prothrombin complex concentrates, intra venous immunoglobulin, immunosuppression, or by extracorporeal removal of the inhibitor. Autoantibody titer, previous response to a given tr eatment, and severity of clinical presentation must be taken into acco unt when choosing between these different therapeutic options, which m ust often be associated.