CAJ | 학술논문

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(HA-FⅧ: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.
목적 탐토혈우병적병인학분류방법.방법 수궤선취2005년2월지2010년5월협서의대혈액병연구원접진적500례혈우병환자위연구대상.대기채용일기법검측응혈인자활성,Bethesda법검측FⅧ、FIX항체,활화부분응혈활매시간(APTT),정상인혈장혼합APTT규정시험,감별응혈인자결핍、동충항체여자신획득성혈우병.안조2000년전국혈전여지혈회의진단표준급본연구제출적선천유전여비유전、후천동충여자신획득성혈우병량류4형병인학분류방법,대수시자진행병인학분류.결과 500례혈우병환자중,선천성혈우병위490례(98％),기중유전성위376례(75.2％)、비유전성위114례(22.8％％),혈우병A(HA)위411례(82.2％)、혈우병B(HB)위79례(15.8％).HA병발FⅧ항체(HA-FⅧ:Ab)위151례(30.2％),HB병발FⅨ항체(HB-FⅨ:Ab)위18례(3.6％)；후천자신획득성혈우병위10례(2.0％),균위다충응혈인자동시결핍.결론 혈우병병인학분류대혈우병적방치,가제공중요의거.
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.