血管性血友病86例临床诊断和治疗分析  

Analysis on clinical diagnosis and management of 86 patients with von Willebrand disease

作　　者：许剑辉[1] 许现辉[1] 陈云飞[1] 李慧媛[1] 薛峰[1] 张磊[1] 杨仁池[1] 

机构地区：[1]中国医学科学院北京协和医学院 血液学研究所血液病医院 血栓及止血中心,天津300020

出　　处：《临床血液学杂志》2013年第6期789-791,共3页Journal of Clinical Hematology

基　　金：协和青年基金资助;中央高校基本科研业务费专项基金资助(No:3332013071)

摘　　要：目的:分析血管性血友病(vWD)的特点,探讨其临床诊断和治疗的影响因素。方法:收集86例vWD患者的病史和实验结果,对不同亚型患者的指标进行比较,并对患者贫血、出血表现及治疗需要的影响因素进行相关性分析。结果:86例vWD患者中,41例诊断为3型vWD,其余45例为未分型vWD。3型vWD患者的因子Ⅷ活性(FⅧ∶C)和瑞斯托霉素诱导的血小板聚集(RIPA)显著低于未分型患者(均P<0.05)。鼻出血难止是患者并发贫血的独立性因素(R=3.22,P<0.05)。vWF抗原水平(vWF∶Ag)和FⅧ∶C与患者的临床出血积分呈显著负相关(R=-0.38,P<0.01;R=-0.26,P<0.05),接受替代治疗的患者vWF∶Ag和RIPA水平均显著低于未接受替代治疗患者(0.01∶0.06,P=0.026;0.085∶0.275,P=0.034)。结论:临床上3型vWD的比例较高提示需要更为完善的诊断策略和手段,鼻出血的防治对于减少vWD患者并发贫血可能有重要的意义,vWF∶Ag和RIPA的显著低下对临床给予vWF替代治疗可能有重要的指示作用。Objective：To explore the factors influencing clinical diagnosis and treatment of von Willebrand dis- ease （vWD）. Method：Clinical data and laboratory records of 86 vWD patients were collected and divided into dif- ferent subtypes. We analyzed the related factors which influenced anemia, bleeding signs and treatment require- ments. Result：Forty-one patients were diagnosed as type 3 vWD and the remaining 45 patients were diagnosed as unclassified vWD. The factor Ⅷ activities （FⅧ ： C） and ristocetin induced platelet aggregation index （RIPA） were obviously lower in type 3 vWD patients than those in unclassified patients. Refractory epistaxis was a separated factor influencing the onset of patients anemia （R= 3.22,P〈0.05）. The vWF antigen and FⅧ ： C levels had ap- parently negative relationship with patients clinical bleeding score （R = - 0.38, P〈0.01 ;R = - 0.26, P〈0.05 re- spectively）. Patients with refractory bleeding and received substitution therapy had much lower vWF ： Ag and RI- PA levels than those who did not receive substitution therapy （0.01 ： 0.06,P=0. 026;0. 085 ： 0. 275,P=0. 034 respectively）. Conclusion：The high rate of type 3 vWD patients suggests the requirement of more sophisticated clinical diagnoses strategy and available laboratory reference. Prophylaxis of or active treatment on epistaxis may prevent anemia complaints. The downward levels of both vWF ： Ag and RIPA have important implies on undertak- ing substitution therapy for vWD patients.

关 键 词：血管性血友病 实验室检测 vWF∶Ag FⅧ∶C 瑞斯托霉素诱导的血小板聚集 

分 类 号：R554[医药卫生—血液循环系统疾病]