Factor VIII inhibitors in hemophilia A: rationale and latest evidence

被引：170

作者：

Witmer, Char ^{[1
,2
]}

Young, Guy ^{[1
]}

机构：

[1] Childrens Hosp Philadelphia, Div Hematol, Civ Ctr Blvd,CTRB 11th Floor,Room 11026, Philadelphia, PA 19104 USA

[2] Childrens Hosp Angeles & Univ So Calif Keck Sch M, Div Hematol Oncol, Los Angeles, CA 19104 USA

来源：

THERAPEUTIC ADVANCES IN HEMATOLOGY | 2013年 / 4卷 / 01期

关键词：

FVIII; FVIII alloantibodies; hemophilia A; immune tolerance; inhibitors; risk factors;

D O I：

10.1177/2040620712464509

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Factor VIII (FVIII) replacement therapy is the foundation of treatment in hemophilia A and is effective unless a patient develops an alloantibody (inhibitor) against exogenous FVIII. Inhibitor development is currently the most significant treatment complication seen in patients with hemophilia and is associated with considerable morbidity and a decreased quality of life. The development of an inhibitor is the result of a complex interaction between a patient's immune system and genetic and environmental risk factors. The mainstay of treatment is the eradication of the inhibitor through immune tolerance. This review summarizes the current evidence regarding inhibitor risk factors, eradication, and hemostatic bypassing agents.

引用

页码：59 / 72

页数：14

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