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
相关论文
共 76 条
[71]  
Toschi V, 2010, CURR OPIN MOL THER, V12, P617
[72]   Recommendations for the implementation of a Patient Blood Management programme. Application to elective major orthopaedic surgery in adults [J].
Vaglio, Stefania ;
Prisco, Domenico ;
Biancofiore, Gianni ;
Rafanelli, Daniela ;
Antonioli, Paola ;
Lisanti, Michele ;
Andreani, Lorenzo ;
Basso, Leonardo ;
Velati, Claudio ;
Grazzini, Giuliano ;
Liumbruno, Giancarlo M. .
BLOOD TRANSFUSION, 2016, 14 (01) :23-65
[73]  
VERBRUGGEN B, 1995, THROMB HAEMOSTASIS, V73, P247
[74]   Diagnostic and prognostic value of factor VIII binding antibodies in acquired hemophilia A: data from the GTH-AH 01/2010 study [J].
Werwitzke, S. ;
Geisen, U. ;
Nowak-Goettl, U. ;
Eichler, H. ;
Stephan, B. ;
Scholz, U. ;
Holstein, K. ;
Klamroth, R. ;
Knoebl, P. ;
Huth-Kuehne, A. ;
Bomke, B. ;
Tiede, A. .
JOURNAL OF THROMBOSIS AND HAEMOSTASIS, 2016, 14 (05) :940-947
[75]   Treatment of acquired hemophilia by the Bonn-Malmo Protocol: documentation of an in vivo immunomodulating concept [J].
Zeitler, H ;
Ulrich-Merzenich, G ;
Hess, L ;
Konsek, E ;
Unkrig, C ;
Walger, P ;
Vetter, H ;
Brackmann, HH .
BLOOD, 2005, 105 (06) :2287-2293
[76]   The relevance of the bleeding severity in the treatment of acquired haemophilia - an update of a single-centre experience with 67 patients [J].
Zeitler, H. ;
Ulrich-Merzenich, G. ;
Goldmann, G. ;
Vidovic, N. ;
Brackmann, H. H. ;
Oldenburg, J. .
HAEMOPHILIA, 2010, 16 :95-101