[后天性血友病 A 的新型治疗策略]。

Satoshi Higasa
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引用次数: 0

摘要

获得性血友病 A(AHA)是一种出血性疾病,由针对血液凝固因子 VIII(FVIII)的自身抗体(抑制剂)产生所致。其特点是突然发病,通常会导致软组织大面积严重出血。当凝血试验显示 PT 正常、APTT 延长、FVIII 活性降低、VWF 活性正常和 FVIII 抑制剂阳性时,即可诊断为获得性血友病 A。止血疗法主要包括旁路疗法,这种疗法可激活外源性凝血途径,从而避开对 FVIII 或因子 IX 的需求。Emicizumab是一种可替代FVIII功能的双特异性抗体,可用于预防出血。免疫抑制疗法是抑制或根除抑制因子的必要手段。大多数患者在接受治疗后病情会得到缓解,但也有一些患者会死于出血症状或与免疫抑制治疗相关的感染。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Novel treatment strategies for acquired hemophilia A].

Acquired hemophilia A (AHA) is a bleeding disorder caused by autoantibody (inhibitor) production targeting blood coagulation factor VIII (FVIII). It is characterized by sudden onset, and often causes extensive and severe bleeding in soft tissue. Acquired hemophilia A is diagnosed when coagulation tests show normal PT, prolonged APTT, decreased FVIII activity, normal VWF activity, and positive FVIII inhibitor. Hemostatic therapy mainly consists of bypass therapy, which activates the extrinsic coagulation pathway, bypassing the need for FVIII or factor IX. Emicizumab, a bispecific antibody that substitutes for FVIII function, can be used to prevent bleeding. Immunosuppressive therapy is necessary to suppress or eradicate inhibitors. The majority of patients go into remission with treatment, but some die from bleeding symptoms or infections associated with immunosuppressive therapy.

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