The genetic basis of inhibitor development in haemophilia A

被引：41

作者：

Tuddenham, EGD ^{[1
]}

McVey, JH ^{[1
]}

机构：

[1] Hammersmith Hosp, Imperial Coll, MRC, Ctr Clin Sci,Sch Med, London W12 0NN, England

来源：

HAEMOPHILIA | 1998年 / 4卷 / 04期

基金：

英国医学研究理事会;

关键词：

haemophilia A; factor VIII; inhibitor; antibodies; mutation;

D O I：

10.1046/j.1365-2516.1998.440543.x

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Inhibitor development is now the main complication of replacement therapy in haemophilia A. Given that most severely affected patients make no detectable factor VIII, it is perhaps surprising that only similar to 30% actually mount an immune response to factor VIII as a foreign antigen. Those that do mostly have major factor VIII gene lesions. The association of HLA genotype with inhibitors in patients with identical mutations is weak. Environmental factors may be more important than genetic in antibody response to factor VIII.

引用

页码：543 / 545

页数：3

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