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血管性血友病因子GPIb活性/抗原比值在血栓性血小板减少性紫癜快速鉴别诊断中的价值 被引量:1

The rapid differential diagnostic value of the von Willebrand factor GPIb activity/antigen ratio for thrombotic thrombocytopenic purpura
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摘要 目的评估血管性血友病因子(vWF)GPIb活性/抗原比值对血栓性血小板减少性紫癜(TTP)急性期的诊断价值。方法前瞻性纳入2018年1月至2019年12月入院的93例同时存在血小板计数<50×10^9/L和溶血表现的疑诊TTP患者,检测ADAMTS13活性,vWF:GPIb活性及vWF抗原,以ADAMTS13活性测定结果作为TTP确诊依据,记录患者PLASMIC临床评分及最终临床诊断。结果22例患者确诊为TTP,ADAMTS13活性的中位数及四分位间距为1.0%(0~7.4%),71例非TTP患者主要诊断为溶血尿毒综合征(HUS)、重症感染、恶性肿瘤、结缔组织病等。TTP患者vWF:GPIb活性、抗原及两者比值均低于非TTP患者(P<0.05),vWF:GPIb活性/抗原比值<0.75对TTP诊断效力最高(敏感度和特异度分别为81.8%、95.2%)。在PLASMIC临床评分判断为TTP高可能性的患者中,vWF:GPIb活性/抗原比值<0.75者有88.9%最终确诊为TTP,比值≥0.75者有15.4%最终确诊为TTP。结论当ADAMTS13活性结果不能及时获得时,基于自动化检测的vWF:GPIb活性/抗原比值有助于疑诊TTP患者的快速鉴别和风险分层。 Objective To evaluate the diagnostic value of the von Willebrand factor(vWF)GPIb activity to antigen ratio for thrombotic thrombocytopenic purpura(TTP)at acute phase.Methods A cohort of 93 patients with suspected TTP and admited to our hospitalfrom January 2018 to December 2019 were prospectively enrolled,we analyzed the levels of vWF:GPIb activity and vWF antigen at acute phase,the diagnosis of acquired TTP was defined as ADAMTS13 activity<10%.The PLASMIC score and final diagnosis of included patients were recorded.Results Twenty-twopatients were diagnosed as TTP,the median(interquartile range)of ADAMTS13 activity were 1.0%(0-7.4%),other diagnoses included hemolytic uremic syndrome(HUS,n=8),severe infection(n=21),malignancy(n=3),connective tissue disease(n=8),et al.The vWF:GPIb activity,vWF antigen,and ratio of vWF:GPIb activity to antigen were all significantly lower in patients with TTP than in those without(P<0.05).The ratio of vWF:GPIb activity to vWF antigen<0.75 could yield a sensitivityof 81.8%and a specificity of 95.2%respectively for TTP.In patients with PLASMIC score≥6,88.9%patients with the ratio of vWF:GPIb activity to vWF antigen<0.75 were confirmed as TTP,and 15.4%patients with the ratio≥0.75 were confirmed as TTP.Conclusions The ratio of vWF:GPIb activity to vWF antigen based on automaticassays might be useful for differential diagnosis and stratification of TTP at acute phase,especially when the ADAMTS13 assay is not available in time.
作者 唐宁 赵倩雯 白欢 李登举 Tang Ning;Zhao Qianwen;Bai Huan;Li Dengju(Department of Clinical Laboratory,Tongji Hospital,Tongji Medical College,Huazhong University of Science and Technology,Wuhan 430030,China;Department of Clinical Laboratory,Affiliated hospital of Guilin Medical College,Guilin 541001,China;Department of Hematology,Tongji Hospital,Tongji Medical College,Huazhong University of Science and Technology,Wuhan 430030,China)
出处 《中华检验医学杂志》 CAS CSCD 北大核心 2020年第8期812-815,共4页 Chinese Journal of Laboratory Medicine
关键词 紫癜 血栓性血小板减少性 von Willebrand因子 ADAMTS13蛋白质 诊断 鉴别 Purpura,thrombotic thrombocytopenic Von willebrand factor ADAMTS13 protein Diagnosis,differential
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