血友病患者抗凝物质及纤溶抑制物质的改变及其在血友病出血机制中的意义  

Change and Significance of Anticoagulants and Fibrinolysis Inhibitor in Patients with Hemephilia

作　　者：胡理明[1] 谢炳寿[1] 朱淼勇[1] 叶玲丽[1] 金小丽[1] 陈萍[1] 周奇[1] 

机构地区：[1]温州市第三人民医院血液科,温洲325000

出　　处：《血栓与止血学》2010年第4期154-157,共4页Chinese Journal of Thrombosis and Hemostasis

摘　　要：目的探讨抗凝物质及纤溶抑制物质在血友病中的改变及其在血友病出血机制中的作用。方法分别对近期未治疗的血友病患者65例(A型54例,B型11例),58例(A型48例,B型10例)及20例(A型15例,B型5例)以ELISA法检测组织因子途径抑制物(TFPI)、蛋白C(PC)、游离蛋白S(FPS),以速率散射比浊法检测抗凝血酶(AT)以及以生色法检测凝血酶激活的纤溶抑制物活性(TAFIa)。其中TFPI、PC、FPS、AT正常健康对照20例,TAFIa正常健康对照组15例。采用SPSS统计软件包进行统计学分析,结果采用t检验。以实验结果为依据对血友病出血机制中抗凝物质及纤溶抑制物质的作用进行探讨。结果血友病患者与正常健康对照血浆抗凝物质及纤溶抑制物质TFPI、PC、FPS、AT及TAFIa值分别为:TFPI96.90±24.06ng/mlvs85.17±12.62ng/ml,P<0.05;PC5.02±1.37μg/mlvs5.34±0.86μg/ml,P>0.05;FPS8.99±3.44μg/mlvs9.17±2.32μg/ml,P>0.05;AT308.27±64.68μg/mlvs236.85±21.73μg/ml,P<0.05;TAFIa86.00±15.67%vs107.00±9.63%,P<0.001。结果显示血友病患者血浆TFPI、AT水平明显高于正常对照,P<0.05;血浆TAFIa水平明显低于正常对照,P<0.001。结论导致血友病出血的凝血酶生成障碍不仅由于FⅧ或FⅨ缺乏,而且与血浆TFPI及AT水平升高,凝血酶生成负调节增强密切相关。提示以抑制TFPI或AT为靶点,有可能为制止血友病出血开辟新途径。血友病患者血浆TAFIa水平降低,下调纤溶机制减弱导致血友病纤溶活性增强,表明抗纤溶措施是血友病治疗的重要环节。Objective To explore the change and significance of anticoagulants and fibrinolysis inhibitor in patients with hemophilia. Methods Plasma levels of factor Ⅷ（FⅧ）,factor Ⅸ（FⅨ） as well as tissue factor pathway inhibitor （TFPI） were measured in 65 patients with hemophilia ,while,protein C （PC）,free protein S （FPS）,antithrombin （AT） were measured in 58 patients with hemophilia and activated thrombin-activitable fibrinolysis inhibitor （TAFIa） were measured in 20 patients with hemophilia. FⅧand FⅨ were measured by one-strage clotting assays;TFPI ,PC ,FPS were measured by ELISA;AT was messured by ratenephelometry and TAFIa was measuned by the synthetic chromogenic substrate method.Results A statistically significant increase were found in plasma levels of TFPI,AT compaed to healthy controls（ TFPI 96.90±24.06 ng/ml vs 85.17±12.62 ng/ml,P〈0.05;AT 308.27±64.68 μg/ml vs 236.85±21.73 μg/ml,P〈0.05）,while a statistically significant decrease were found in plasma levels of TAFIa compared to healthy controls （86.00 15.67% vs 107.00 9.63%,P〈0.001）.No differences in the mean levels of PC and FPS were observed between the patients with hemophilia and healthy controls.Conclusions The results indicate that the bleeding cause in patients with hemophilia is not only absence of FⅧ or FⅨ but also relative to increase of levels of TFPI,AT and decrease of level of TAFIa.

关 键 词：血友病 抗凝物质TFPI、PC、PS、AT 纤溶抑制物质 

分 类 号：R554.1[医药卫生—血液循环系统疾病]