文档介绍：血栓性血小板减少性紫癜和血栓性微血管病诊治指南TTP的诊断thefullpentad:thrombocytopenia,MAHA,fluctuatingneurologicalsigns,renalimpairmentandfever40%ofthepatientsdeveloppentadupto35%ofpatientswithoutneurologicalsignsclinicalhistory,examinationofthepatientandthebloodfilmADAMTS13assayshelptoconfirmthediagnosisandmonitorthecourseofthedisease,notnecessaryforthediagnosis完善TTP诊断的相关检查TTP的预后untreatedmortality—90%afterPEXmortality—20%urredwithin24hofpresentationADAMTS13assaysADAMTS13activity(<5%)±thepresenceofIgGantibodiesconfirmsthediagnosisADAMTS13activity(<40%but>5%)inawidevarietyofnon-TTPconditionssuchasuraemia,inflammatorystates,post-mendationofdiagnosis thegeneralruleTTPshouldbetreatedasamedicalemergency(1A)TheinitialdiagnosisofTTPshouldbemadeonclinicalhistory,examinationandroutinelaboratoryparametersofthepatient,includingbloodfilmreview(1A)PEXshouldbeinitiatedassoonaspossible,preferablywithin4–8h(1A)mendationofdiagnosis thegeneralruleSerologicaltestsforHIV,hepatitisBvirus,hepatitisCvirusandpregnancytestshouldbeperformedatpresentation(1A)Pre-treatmentsamplesshouldbeobtainedtomeasureADAMTS13activitylevelsandtodetectanti-(1B)mendationoftreatmentofTTP ,usingS/Dplasmainallagegroupsandreassesseddaily(1B).(2C).IntensificationinfrequencyandorvolumeofPEXproceduresshouldbeconsideredinlife-threateningcases(2B).DailyPEXshouldcontinueforaminimumof2dafterplateletcounthasbeen>150×109/Landthenstopped(2B).SubgroupsofTTPCongenitalTTP(Upshaw-SchulmanSyndrome)AcuteidiopathicTTPHIV-associatedTTPPregnancy-associatedTTPDrug-associatedTTPPancreatitis-associatedTTP