L’hémophilie A acquise : savoir y penser

Abstract

AbstractAcquired hemophilia A (AHA) is a rare, potentially serious bleeding disorder caused by antibodies directed against factor VIII (FVIII). It is most often diagnosed in older individuals, regardless of sex, and in half of cases there are comorbidities (autoimmune disease, cancer). Acquired hemophilia was also described in women during pregnancy or in post-partum. Mortality remains high, due to underlying comorbidities, bleeding and iatrogenic complications, as well as delayed diagnosis and management. When faced with an unusual, spontaneous bleeding syndrome, the diagnosis of AHA must be considered in the presence of an isolated prolongation of the partial thromboplastin time, and confirmed by the demonstration of an acquired FVIII deficiency and the presence of a specific FVIII inhibitor. In the presence of anticoagulant therapy, biological diagnosis could be difficult. Treatment of AHA involves controlling bleeding and eradicating the inhibitor. Hemostatic treatment is only indicated in cases of severe bleeding or invasive procedures. It relies on inhibitor short-circuiting agents such as recombinant activated factor VII or activated prothrombin complex factor concentrate. Immunosuppression is recommended for all patients, according to international recommendations published in 2020.