获得性血友病A：近期数据及新治疗选择综述
Acquired hemophilia A: a review of recent data and new therapeutic options.
作者信息
Franchini Massimo, Vaglio Stefania, Marano Giuseppe, Mengoli Carlo, Gentili Sara, Pupella Simonetta, Liumbruno Giancarlo Maria
机构信息
a Italian National Blood Centre, National Institute of Health , Rome , Italy.
b Department of Haematology and Transfusion Medicine , 'Carlo Poma' Hospital , Mantua , Italy.
出版信息
Hematology. 2017 Oct;22(9):514-520. doi: 10.1080/10245332.2017.1319115. Epub 2017 Apr 25.
OBJECTIVES
Acquired hemophilia A (AHA) is a rare, but potentially life-threatening, bleeding disorder caused by an autoantibody against factor VIII that interferes with its coagulant function.
METHODS
We performed a narrative review focusing on the diagnostic aspects of AHA and on the current treatment strategies with particular regard to new data and therapeutic developments.
RESULTS
The management of this severe hemorrhagic disorder is based on the control of bleeding with the use of bypassing agents and on the utilization of a variety of immunosuppressant agents with the goal of eliminating the autoantibody permanently.
CONCLUSION
The optimal management of AHA should be multidisciplinary and requires a close collaboration between physicians from various specialties.
目的
获得性血友病A（AHA）是一种罕见但可能危及生命的出血性疾病，由针对凝血因子VIII的自身抗体引起，该抗体干扰其凝血功能。
方法
我们进行了一项叙述性综述，重点关注AHA的诊断方面以及当前的治疗策略，特别关注新数据和治疗进展。
结果
这种严重出血性疾病的管理基于使用旁路制剂控制出血以及使用多种免疫抑制剂以永久消除自身抗体。
结论
AHA的最佳管理应是多学科的，需要各专业医生之间密切合作。
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