500例血友病的病因学分类研究

Etiological Classification for Hemophillia in 500 Cases

目的探讨血友病的病因学分类方法。方法随机选取2005年2月至2010年5月陕西医大血液病研究院接诊的500例血友病患者为研究对象。对其采用一期法检测凝血因子活性，Bethesda法检测FⅧ、FⅨ抗体，活化部分凝血活酶时间（APTT），正常人血浆混合APTT纠正试验，鉴别凝血因子缺乏、同种抗体与自身获得性血友病。按照2000年全国血栓与止血会议诊断标准及本研究提出的先天遗传与非遗传、后天同种与自身获得性血友病两类4型病因学分类方法，对受试者进行病因学分类。结果500例血友病患者中，先天性血友病为490例（98％），其中遗传性为376例（75．2％）、非遗传性为114例（22．8%），血友病A（HA）为411例（82．2％）、血友病B（HB）为79例（15．8％）。HA并发FⅧ抗体（HAFⅧ：Ab）为151例（30．2％），HB并发FⅨ抗体（HB-FⅨ：Ab）为18例（3．6％）；后天自身获得性血友病为10例（2．0％），均为多种凝血因子同时缺乏。结论血友病病因学分类对血友病的防治，可提供重要依据。

Objective To study the etiological classification method in hemophillia. Methods From February 2005 to May 2010, 500 hemophillia patients were included in this study in Shaanxi Medical University Hematology Institute. The activity of coagulation factor was tested by one stage assay. The antibodies of FⅧ and FⅨ were tested by Bethesda assay. According to the diagnostic criteria of thrombosis and homeostasis conference in 2000, the etiology was classified as four types, as flowers： Congential hereditary hemophilia, congential non-hereditary hemophilia, acquired allogeneic hemophilia and acquired autogeneic henophillia. Results The congential hemophillia were 490（98%） cases, of which 376 （75.2%） cases were hereditary, 114（27.8%）cases were non-hereditary, 411（82.2%） cases were hemophillia A and 79（15. 8%） cases were Hemophillia B. Hemophillia A associated with FⅧ antibody were 151 cases （30.2%）, hemophillia B combined with FIX antibody were 18 cases （3.6%）. There were 10 cases （2.0%） of acquired autogeneic hemophilia. All of them were multiple coagulation factors deficient. Conclusion This study recommended an important basis for the etiologic classification of hemophillia.