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

来源：

HEMATOLOGY | 2017年 / 22卷 / 09期

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

页码：514 / 520

页数：7

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