摘要
目的随访研究中国血友病A患者因子Ⅷ(FⅧ)抑制物发生率和特征。方法 215例血友病A患者在24月(2007年6月至2009年6月)的连续随访中,监测FⅧ抑制物发生、变化及转归,并观察患者临床特征。结果 215例血友病A患者随访24月FⅧ抑制物累积发病率为11.6%(25/215);其中低滴度者占72%(18/25),高滴度者占28%(7/25);FⅧ抑制物阳性发生时的中位年龄为25岁(6～59岁)、累积中位暴露日为150日;15/25(60%)低滴度阳性者(中位滴度1.25BU/ml)在自然情况下于6～15月(中位10月)转为阴性,5/25(20%)高滴度抑制物者(中位滴度100BU/ml)则随访24月持续阳性,另外5/25(20%)FⅧ抑制物阳性无变化;25例FⅧ抑制物阳性者出血频率较其阴性时显著增加(P=0.025);18/25例继续应用FⅧ者,FⅧ产品用量(IU/kg·月)较前显著增加(P=0.015),但靶关节数目在24月随访期间并无增加(P=0.329)。结论我国血友病A患者FⅧ抑制物发病率和特征与欧美等国家存在差异。
Objective To study the incidence and characteristics of coagulation factor VIII （FⅧ） inhibitor development in Chinese patients with hemophilia A. Methods A 24-month continuous follow-up was conducted among 215 Chinese patients with hemophilia A to observe the characteristics of FⅧ inhibitor development and the clinical characteristics of the patients. Results The cumulative incidence of FVIII inhibitor development in 24 months was 11.6% （25/215） in these patients. Of the 25 patients with FⅧ inhibitor development,18 （72%） had low-titer inhibitors and 7 （28%） had high-titer inhibitors. The patients developed the inhibitors after a median of 150 exposure days at a median age of 25 years （6-59 years）. Fifteen patients with low-titer inhibitors （median 1.25 BU/ml） showed gradual disappearance of the inhibitors in a median of 10 months （6-15 months） without any treatment,and 5 patients with high-titer inhibitors （median 100 BU/ml） remained positive in 24 months; the other 5 FⅧ inhibitor-positive cases showed no significant changes. In the 25 patients developing FⅧ inhibitors,the bleeding frequency increased significantly （P=0.025）,and in 18 of the patients who continued to use FⅧ products,a significant increase in the dose of medication was noted （P=0.015）,but the number of target joints did not increase in 24 months （P=0.329）. Conclusion The incidence and characteristics of factor Ⅷ inhibitor development differ between Chinese patients with hemophilia A and those in developed countries.
出处
《南方医科大学学报》
CAS
CSCD
北大核心
2010年第12期2721-2724,共4页
Journal of Southern Medical University
基金
诺和诺德血友病基金会(NNHF)中国一期项目
关键词
血友病A
凝血因子Ⅷ
抑制物
发病率
hemophilia A
coagulation factor Ⅷ
inhibitor
incidence
