Acquired hemophilia A: a review of recent data and new therapeutic options

被引:85
作者
Franchini, Massimo [1 ,2 ]
Vaglio, Stefania [1 ,3 ]
Marano, Giuseppe [1 ]
Mengoli, Carlo [1 ]
Gentili, Sara [1 ]
Pupella, Simonetta [1 ]
Liumbruno, Giancarlo Maria [1 ]
机构
[1] Natl Inst Hlth, Italian Natl Blood Ctr, Rome, Italy
[2] Carlo Poma Hosp, Dept Haematol & Transfus Med, Mantua, Italy
[3] Sapienza Univ Rome, Dept Clin & Mol Med, Rome, Italy
关键词
Acquired hemophilia A; factor VIII; bleeding; eradication therapy; FACTOR-VIII INHIBITORS; ACTIVATED FACTOR-VII; TRANSFUSION MANAGEMENT; EXTRACORPOREAL IMMUNOADSORPTION; COAGULATION INHIBITORS; RANDOMIZED-TRIAL; PORCINE SEQUENCE; RECOMBINANT; RECOMMENDATIONS; DIAGNOSIS;
D O I
10.1080/10245332.2017.1319115
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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.
引用
收藏
页码:514 / 520
页数:7
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