获得性血友病 A 的免疫抑制治疗研究进展.

Acquired hemophilia A（AHA) is a rare bleeding disorder caused by the circulating autoantibodies directedly against coagulation factor Ⅷ, resulting in decreased activity of coagulation factor Ⅷ.In recent years, awareness of the disease has gradually increased due to the increased reporting of the disease and data reported from several large registry studies in China and abroad, but due to the relative rarity of the disease itself, there is still a lack of awareness and delay in diagnosis and treatment.Its treatment consists of three main aspects: control of bleeding in the acute phase, eradication of inhibitors, and treatment of the primary disease or removal of the causative agent. Immunosuppressive treatment（IST) to eradicate inhibitors is the therapeutic foundation. However, recent studies have shown that IST-related mortality exceeds even that caused directly by bleeding, so the IST of AHA is still very challenging and requires continuous experience and exploration of optimal treatment protocols. In this paper, we will focus on the basic clinical features, characteristics of coagulation factor Ⅷ inhibitors in acquired hemophilia A, the current major immunosuppressive treatment options, and the latest treatment options. [ABSTRACT FROM AUTHOR]