Acquired hemophilia A: a review of recent data and new therapeutic options
Acquired hemophilia A: a review of recent data and new therapeutic options
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DOI：
10.1080/10245332.2017.1319115
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发表时间：
2017-01-01
期刊：
影响因子：
1.9
通讯作者：
Liumbruno, Giancarlo Maria
中科院分区：
文献类型：
--
作者：
Franchini, Massimo;Vaglio, Stefania;Liumbruno, Giancarlo Maria
目的：获得性血友病A（AHA）是一种罕见的，但可能危及生命的，出血性疾病引起的自身抗体对因子VIII，干扰其凝血功能。方法：我们进行了一个叙述性审查，重点是诊断方面的AHA和目前的治疗策略，特别是关于新的数据和治疗的发展。结果：这种严重的出血性疾病的管理是基于使用旁路剂控制出血和利用各种免疫抑制剂，目的是永久消除自身抗体。结论：AHA的最佳管理应该是多学科的，需要来自不同专业的医生之间的密切合作。
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.