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Abstract Acquired haemophilia is a potentially life threatening bleeding disorder caused by the development of autoantibodies against coagulation factor VIII. In around half of patients, an underlying disorder is associated with AHA; the remaining cases are idiopathic. AHA presents with bleeding, ranging from mild to life threatening, in patients with no personal or family bleeding history. Diagnosis involves an isolated prolongation of the activated partial thromboplastin time, without correction in mixing studies, low FVIII activity levels and evidence of a FVIII inhibitor. As AHA is rare, a lack of familiarity of the condition may result in delayed diagnosis Prompt hemostatic control is required to reduce morbidity and mortality. Bypassing agents (recombinant activated factor VII or activated prothrombin complex concentrates) can be used to control acute bleeding Immunosuppression is necessary to eradicate the inhibitor. Prognosis in AHA depends on presence of comorbidities, on severity of bleeding and on the time to diagnosis and initiation of appropriate anti hemorrhagic and immunosuppressive treatment. As clinical trials in this rare and heterogeneous disease are difficult, current evidence comes from observational studies, including registries. |