후천성 혈우병 A

Abstract

Acquired hemophilia A is rare but fatal bleeding disorder caused by an autoantibody against factor VIII. Acquired hemophilia A is more prevalent in the elderly patients and associated with other underlying diseases such as autoimmune disorders, malignancies, pregnancy, infection and several drugs. The bleeding pattern of acquired hemophilia A is quite different from that of congenital hemophilia A. Thus, acquired hemophilia A should be suspected in the presence of bleeding with sudden onset, often severe, which occurs spontaneously or after minor trauma, following invasive procedures or surgical interventions in patients without a personal or family history of bleeding. The main goal of the treatment for acquired hemophilia A should be the control of bleeding with the use of bypassing agents and the eradication of autoantibodies with the use of immunosuppressive agents. Because the remained level of factor VIII is not correlated with the risk of fatal bleeding, appropriate hemostatic management should be applied in patients with clinically significant bleeding regardless of the level of factor 8 or the inhibitor level of factor VIII. Because current recommendations for acquired hemophilia A are developed mainly based on the retrospectively available data, larger multicenter prospective trials should be conducted for developing appropriate treatments.