血友病凝血因子抗体的诊断及影响因素研究
ZHAO XIAO-Ai,LIU Shaan-xi,LI Fei,LIU Yi-Guo,REN Yong-Ping Department oF Oncology,Xi'an jiaotong University First Affiliated Hospital,Xi'an 710061,ShaanXi Province,China;Institute of Hemalologic Diseases,
Shaanxi Medical University, Xi'an 710061, Shaanxi Province,China
Abstract This study was purposed to investigate the diagnosis,typing ang in fluencing factors of the antibody(inhibitor)to coagulation factors in hemophilia.500 hemophillia patients were enrolled in this study.The activities of coagulation factor FⅧ and FⅨ were tested by one stage assay.The antibodies of FⅧ and FⅨ were detected by bethesda assay.All date were analyzed by statistical soft SPSS 10.0.The results indicated that there were 411cases of Hemophilia A,out of which 151cases(30.2%)showed FⅧ antibody positive,the titer was (3.50±2.84)Bu/mL;There were 79 cases of hemophilia B,out of which 18 cases(3.6%)showed FⅨ antibody positive,the titer was 2.92 ± 2.19Bu/mL.The other 10 cases were acquired autogeneic hemophilia(2.0%).The antibody was divided into three types:high-response 3cases, intermediate-response 47 cases, and lowresp onse 119 cases.Among the 169cases with antibody positive,157 cases(92.9%)were younger than 30 years old;among 151 cases(89.35%)showed intermediate or cases hemophilia A;138 cases(81.66%)were moderate or severe hemophilia;166 case(98.22%)showed intermediate or low-response antibody.there were 158cases with allogeneic antibody positive,all of which received blood transfusion.It is concluded that the moderate and low responsive antibodies are the dominant in hemophilia patients,the age of patients and trans fusion frequency of blood preparation are the influencing factors.The results of this study provide the basis for the hemophilia diagnosis,antibody typing and evaluation of factors influencing hemophilia,and also suggest that the repeated tran -sfusion of blood preparation may influence the production of antibodies。(https://www.daowen.com)
Key words hemophilia;coagulation factor antibody;diagnosis;typing;influence factor
出血是血友病生之具有、伴随终身的主要症状和致残、致死的主要原因。自1940年美国哈佛大学Cohn发明低温乙醇法提取凝血因子防治血友病出血以来,大大减轻了血友病的出血症状,但随之而来的并发症是同种特异性凝血因子抗体(抑制物)的凝血因子活性则会加重出血和提高死亡率。同年(1940年)Lozner等[1]虽报道了获得性血友病,但未引起重视。直到2004年WHO宣布删除人FⅧ、FⅨ浓缩剂计划,则全世界医务工作者方予以真正关切,在此情况下血友病凝血因子抗体的研究得到了开展,本研究探讨凝血因子抗体的诊断分型及其影响因素。