Etiological Classification for Hemophilia in 500 C...
LIU Shaan-xi,LIU Yi-guo,MU Ling-feng,YANG Li-l i,TIAN Wen-hui,LI Juan-juan,DENG Ya-ning Shaanxi Medical University Hematology Institute Xi'an 710061
Abstract Objective To study the etiological classification method in Hemophilia.Methods From February 2005 to May 2010,500 Hemophilia 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 etiolo-gy was classified as four types,as flowers:congenital hereditary hemophilia,congenital non-here ditary hemophilia,acquired allogeneic hemophilia and acquired autogeneic Henophilia.Result The congenital hemophilia were 490(98%)cases,of which 376(75.2%)cases were hereditary, 114(27.8%)cases were non-hereditary, 411(82.2%)cases were hemophilia A and 79(15.8%)cases were Hemophilia B.Hemophilia A associated with FⅧ antibody were 151 cases(30.2%),hemophilia B combined with FIX antibody were 18 cases(3.6%).There were 10 cases(2.0%)of acquired autogenic hemophilia.All of them were multiple coagulation factors deficient.Conclusion This study recommended an important basis for the etiologic classification of hemophilia(https://www.daowen.com)
Key Words hemophilia;etiology;classification
血友病(hemophilia)又称为“皇室病”,一直被认为是一种单纯性联隐性遗传性疾病。近10年来,由于分子生物学和基因技术的发展,发现该病不仅包括遗传性血友病,还存在非遗传性和后天获得性血友病[1-2]。Liu等[3]通过对陕西医大血液病研究院2005年2月至2010年5月收治的500例血友病患者的研究发现,血友病除存在遗传性、非遗传性和后天获得者外,尚有单一凝血因子缺乏和多种凝血因子缺乏。这为该病的临床诊断带来困惑。为规范血友病的诊断与病因学分类,本研究开展对血友病病因学分类专项研究,旨在对血友病的防治,可提供重要依据。现将研究结果,报道如下。