血友病A抑制物产生的危险因素及相关治疗进展

陈昆; 张建华; 王刚; 张夏林; 杨林花

doi:10.13201/j.issn.1004-2806-b.2018.12.023

血友病A抑制物产生的危险因素及相关治疗进展

- 山西医科大学第二医院血液科(太原, 030001)
基金项目:
国家自然科学基金青年科学基金项目(No:81700182)

山西省应用基础研究项目(No:201601D202094)

详细信息

通讯作者: 杨林花,E-mail:Yanglh5282@163.com

中图分类号: R554.1

收稿日期: 2018-09-22

Risk factors in inhibitors development of hemophilia A and advances treatment

More Information

Corresponding author: , E-mail: Yanglh5282@163.com

Received Date: 22 September 2018

摘要

- 血友病A /
- 抑制物 /
- 危险因素 /
- 治疗
Abstract: Haemophilia A（HA）is an X-linked and inherited bleeding disorder which is resulted from deficiency of coagulant factorⅧ（FⅧ）.The severity of hemophilia is classified based on plasma levels of FⅧ activity:severe if＜1%,moderate if between 1 and 5%and mild if＞5 and＜40% of normal.Inhibitor formation is among the most severe complications of hemophilia treatment,with a cumulative incidence of 30%in HA patients.And it usually occurs on the 9 th-12 th exposure day.Inhibitors patients become unresponsive to standard factor treatment,resulting in 2-fold the hospitalizations,10-fold the cost,and 3.5-fold mortality of noninhibitor patients.Furthermore,inhibitors are a recognized contraindication to gene therapy.
- hemophilia A /
- inhibitors /
- risk factors /
- treatment

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