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获得性血友病 A。

Acquired Hemophilia A.

机构信息

McMaster University, Hamilton, Canada; Hamilton Health Sciences, Hamilton, Canada; Hamilton Regional Laboratory Medicine Program, Hamilton, Canada.

出版信息

Hematol Oncol Clin North Am. 2021 Dec;35(6):1131-1142. doi: 10.1016/j.hoc.2021.07.007. Epub 2021 Sep 15.

Abstract

Acquired hemophilia A is a potentially severe bleeding disorder caused by antibodies against the patient's own factor VIII. Acquired hemophilia A is rare. It is most commonly diagnosed in older individuals; about one-half of cases of acquired hemophilia are associated with underlying conditions, including autoimmune disease, cancer, and pregnancy. The diagnosis of acquired hemophilia A can be suspect with an isolated activated partial thromboplastin time elevation, and confirmed with demonstration of reduced factor VIII activity and the presence of a specific factor VIII inhibitor. Treatment of acquired hemophilia A involves control of bleeding, and eradication of the inhibitor.

摘要

获得性血友病 A 是一种由针对患者自身因子 VIII 的抗体引起的潜在严重出血性疾病。获得性血友病 A 较为罕见。它最常发生于老年人;约有一半的获得性血友病病例与潜在疾病相关,包括自身免疫性疾病、癌症和妊娠。孤立的活化部分凝血活酶时间升高即可怀疑获得性血友病 A 的诊断,通过证实因子 VIII 活性降低和存在特定的因子 VIII 抑制剂即可确诊。获得性血友病 A 的治疗包括控制出血和消除抑制剂。

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