获得性血友病诊治进展

Acquired hemophilia: progress in the diagosis and treatment

获得性血友病(AH)是因体内产生针对凝血因子的自身抗体而导致严重的、甚至致命性出血的自身免疫性疾病,其中以凝血因子Ⅷ(FⅧ)自身抗体(抑制物)导致的获得性血友病A(AHA)最为常见。约一半的AHA患者有基础疾病。治疗重点是使用旁路途径药物控制出血以及使用免疫抑制剂清除抗体。在AHA的诊治过程中遇到的挑战主要有诊断延误、出血不易控制、病情不能持续缓解以及治疗药物带来的并发症。

Acquired hemophilia A（AHA） is the most common disorder among autoimmune diseases, which is caused by an autoantibody （inhibitor） to factor Ⅷ （FⅧ） that interferes with the coagulant function and predisposes to severe, potentially life- threatening hemorrhage. In around half of patients, an underlying disorder is associated with AHA; the remaining cases are idiopathic. Disease management focuses on controlling bleeding, primarily with the use of bypassing therapy and recombinant porcine FⅧ, and permanently eradicating the autoantibody using various immunosuppressants. Treatment challenges include delayed diagnosis, difficulty achieving hemostasis and durable remissions, and complications associated with the use ofhemostatic and immunosuppressive therapy.