2A型血管性血友病的临床诊断  

Clinical Diagnosis of the Type 2A Von Willebrand Disease

作　　者：王迎春[1] 张威[1] 程大卫[1] 张敬宇[1] 阮长耿[1] 

机构地区：[1]苏州医学院附属第一医院血栓与止血研究室,江苏省血液研究所苏州215006

出　　处：《苏州医学院学报》2000年第7期630-632,共3页Acta Academiae Medicinae Suzhou

基　　金：国家自然科学基金资助课题!(39670331)

摘　　要：目的　探讨 2A型血管性血友病的临床特征和诊断方法。方法　用出血时间 (BT)、血浆凝血酶原时间(PT)、全血凝固时间 (CT)、活化的部分凝血活酶时间 (APTT)、vWF :Ag、FⅧ :C、FⅧC :Ag、瑞斯脱霉素诱导的血小板凝集试验和血浆多聚物分析 ,对 12 6例有先天性出血倾向的患者进行了研究。结果　确诊为 2A型血管性血友病 14例。皆有初期止血障碍的出血特点 ,表现为BT延长 ,PT、CT正常 ,APTT轻度延长 ,vWF :Ag下降 ,FⅧ :C、FⅧC :Ag轻度下降 ,瑞斯脱霉素诱导的血小板聚集明显降低。血浆中缺乏大中分子量的多聚物。 结论　BT、CT、PT、APTT、vWF :Ag、FⅧ :C、瑞斯脱霉素诱导的血小板聚集试验可作为血管性血友病的初筛试验 。Objective To study the clinical character and diagnosis of type 2A von Willebrand disease (vWD).Methods One hundred and twenty six patients with congenital hemorrhage disease were studied with bleeding time (BT),prothrombin time (PT),clotting time (CT),activated partial thromboplastin time (APTT),vWF:Ag,FⅧ:C,FⅧC:Ag,ristocetin induced platelet aggregation and vWF multimers analysis in plasma.Results Fourteen patients were diagnosed as the type 2 vWD.The common characters of abnormal initial stage hemostasis existed in these patients.They presented prolonged BT,natural PT and CT,slightly prolonged APTT,reduced vWF:Ag,FⅧ:C and FⅧC:Ag,absented high and intermediate molecular weight forms of vWF multimers in plasma and markedly reduced platelet aggregation in response to ristocetin.Conclusions BT,CT,PT,APTT,vWF:Ag,FⅧ:C and platelet aggregation in response to ristocetin is considered of the initial test to diagnosed the type 2A vWD.The vWF multimes analysis has more significance to diagnose this type.

关 键 词：2A型血管性血友病 临床特点 诊断 

分 类 号：R554.1[医药卫生—血液循环系统疾病]