血管性血友病四种指标实验检测意义的比较

The comparison study of four kinds of tests for diagnosis of von Willebrand disease

目的探讨血管性血友病4种检测实验诊断方法的临床应用价值。方法使用vWF抗原水平检测(vWFAg)、vWF胶原结合分析实验(vWFCBA)、瑞斯托霉素辅因子活性测定(vWFRcof)、瑞斯托霉素诱导的血小板聚集试验(RIPA)4种方法同时对正常献血员、vWD患者及其他出血性疾病患者进行检测,比较其检测方法的优越性。结果4种检测血管性血友病的实验其结果在vWD患者组与正常献血员组和其他出血性疾病组比较P<001,差异均有统计学意义;正常献血员组与其他出血性疾病组比较P>005,差异无统计学意义。1型vWD患者组中4种检测方法相关性比较显示,vWFCBA与vWF∶Ag相关性最好(r=09610),其次为vWF∶Rcof与RIPA(r=09164),而vWF∶Ag与RIPA相关性最差(r=08132)。vWF∶CBA的变异系数(39%)最小,vWF∶Ag(41%)次之,而vWF∶Rcof和RIPA较高(分别为155%和173%)。4种检测方法与诊断的总符合率分别为vWF∶Ag857%,vWF∶Rcof762%,RIPA809%,vWF∶CBA952%。结论vWF∶CBA操作简便、重复性较好,在常规vWD的诊断分型中可替代vWF∶Rcof和RIPA。

Objective To study the clinical application of four kinds of tests for von Willebrand disease (vWD).Methods The von Willebrand factor collagen binding assay (vWF:CBA), von Willebrand factor antigen levels ( vWF:Ag), standard ristocetin cofactor(vWF:Rcof)and ristocetin induced platelet aggregation (RIPA) were mearsured from healthy donors, patients with different types of vWD, hemophilia and other bleeding disorders. Comparing the four kinds of assays for their ability to detect vWD.Results In general, the four assays have the capability to identify vWD from normal and other bleeding disorders and can be used for diagnosing vWD. In type 1 vWD group, vWF:CBA and vWF:Ag showed a correlation(r=0.9610),followed by vWF:Rcof and RIPA (r=0.9164), vWF:Ag and RIPA showed the lowest (r=0.8132). The coefficient of variation of vWF:CBA was the smallest (3.9%), followed by vWF:Ag (4.1%), the coefficient of variation of vWF:Rcof and RIPA were higher (15.5% and 17.3%, respectively).The concordance of the four assays on vWD was vWF:Ag 85.7%,vWF:Rcof 76.2%,RIPA 80.9%,vWF:CBA 95.2% ,respectively.Conclusion vWF:CBA measured by ELISA is highly reproducible, and easy to operate. The measurement of the functional activity of vWF by vWF:Rcof or RIPA can be replaced by the more reliable vWF:CBA.