92例血友病临床分析及相关传染病调查

【摘要】目的对血友病A和B患者从临床症状、实验室检查、相关传染病进行分析。方法92例血友病患者，74例血友病A，18例血友病B，从发病年龄、出血部位、家族史、测其血小板数、PT、aPTT、TT、FⅧ：C、FⅨ：C、因子抑制物、乙肝二对半、HCV、HIV。结果血友病A和B以及关节出血，皮下血肿发生，多自家族史，aPTT明显延长，88.3±42.84和79.90±31.68s，血友病AFⅧ：CF17.25±8.24%，血友病B3.83±3.64%，FⅧ抑制物4.1%，血友病B未见HIV出现。结论血友病A发病率大于血友病B为4.1∶1;血友病A较血友病B易产生抑制物;在治疗过程中，应注意相关传染病的传播

【关键词】血友病A、B;凝血因子Ⅷ、Ⅸ活性

ClinicalAnalysisandRelatedInfectionDiseaseSurveyof92PatientswithHemoppilia

Abstract:ObjectiveToanalysisthepatientswithhemophiliaAandBinclinicalsymptoms,laboratorytestsandrelaletinfectiondiseases.Methods:Theplatelet,prothrombintime（PT）,activatedpartialthromboplastintime（aPTT）,thrombintime（TT）,factorⅧ:coagulant（FⅧ:c）,factorⅪcoagulant（FⅪ:C）,factorⅧ:inhibitor,markofhepatitisBandCvirus（HBV,HCV）andhumanimmunodeficiencyvirus（HIV）weretestedin92patientswithhemophilia.（72patientswithhemophiliaAand18patientswithhemophiliaB）.ResultsaPTTwereimportantlonger88.3±42.84sand77.90±31.68srespectiveinthepatientswithhemophiliaAandB;theFⅧ:Cwere17.25±8.24%inthepatientswithhemophiliaAandFⅪCwere3.83±3.64%inthepatientswithhemophiliaB.ThefactorⅧinhibitorsappeared4.1%.NoneinhibitorinhemophiliaB.ThepositiveratesofHCVwere6.7%and5.5%respectiveinthepatientswithhemophiliaAandB.NoneHIV.Conclusion:TheincidencerateofhemophiliaAwashigherthanhemophiliaB（4.1∶1）.TheinhibitorofhemophiliaAwasmorethanhemophiliaB.Inthetreatment,weshouldattentedtoconcernsregardingtheriskofviraltransmission.

Keywords:HemophiliaAandB;FⅧ:CandFⅪ:C

血友病A和B是常见的伴性隐性遗传性出血性疾病。是由于血浆内缺乏凝血因子Ⅷ和Ⅸ而出现自发性出血和外伤后出血不止。发生率约3~4/10万人口。男性发病，终生需要输注凝血因子Ⅷ或Ⅸ、新鲜冰冻血浆、冷沉淀物，但会有传播传染病的危险［1,2］。本文对广东地区，我院就诊的92例血友病人临床症状，实验室检查、治疗及HCV、HIV感染进行分析［3,4］。