《易栓症神内病例》PPT课件

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1、易栓症Normalhemostasis（止血）VIIVIITF-VIIaXXaIIIIaFbgnFibrinsIXTFFibrinpVIIIVTFTF-VIIaIXaadhesion粘附aggregation聚集coagulationpathway凝血途径Coagulationbalanceanticoagulantmechanismsprocoagulantmechanismsfibrinformation(VIIa)Xa,IXaCa2+VIIaIIaTM+IIaVIIaIIaCoagulationwaterfallPrecallicreinaXIIXIIaXIXIaIXIXaTFXXaV

2、IIIaVaIIIIaFibrinclotXIIIaXIIIChininogenProteinCAPCPSInhibitionIIaVVIIaVIICallicreinFibrinogenPlasminogenPAI-1PlasminVIIITFPIAntithrombinAPCinhibitorCa2+Ca2+Ca2+Ca2+FibrinmonomerEndothelialcellsurfacecoagulationfactorsanticoagulantproteinsgain-of-functionmutationsfibrinolysisantiphospholipidAbfibri

3、nformationanticoagulantmechanismsprocoagulantmechanisms易栓症（Thrombophlilia）指由于抗凝蛋白、凝血因子、纤溶蛋白等的遗传性或获得性缺陷，或存在获得性危险因素，而容易发生血栓栓塞的疾病状态。不是单一的疾病。血栓栓塞类型：静脉血栓栓塞AnnualincidenceofDVTWhites0.8-1.2x1000HongKongChinese0.16x1000Liuetal,HongKongMedJ2002rateratio(vswhite)AfricanAmericans1.27(1.07-1.51)Hispanic0.60(0

4、.54-0.67)Asians/PacificIslanders0.26(0.22-0.30)*Whiteetal,AnnInternMed1998*idiopathicorsecondaryVTE:amultifactorialdiseaseacquiredgeneticmixedtransientVTEriskfactors…theoldstory.Geneticriskfactorsinheritedthrombophiliadeficiencyofanticoagulantproteinsyearofdiscoveryantithrombin1965proteinC1981prot

5、einS1984ANTITHROMBIN,PROTEINC,PROTEINS:typeofdeficiencyTypeI:QuantitativedeficiencyTypeII:Qualitativedeficiency↓antigenictest↓functionaltest↓functionaltest=antigenictestPrevalenceofdeficienciesgeneralunselectedpopulationVTEpatientsantithrombin0.02-0.2%1%proteinC0.1-0.5%3%proteinS?1-2%RiskofVTEasso

6、ciatedwithdeficienciesincreaserelativeriskantithrombin5-50proteinC7-15proteinS6-10GeneticriskfactorsinheritedthrombophiliayearofdiscoveryresistancetoactivatedproteinC1993/94andfactorVLeiden(G1691A)prothrombinmutation(G20210A)1996…thenewstory.…theoldstory.Acquiredriskfactorsoftenpersistent(unremova

7、ble)olderagecancerantiphospholipidantibodiespreviousvenousthromboembolismAgeandVTE0-910-1920-2930-3940-4950-5960-6970-79>796005004003002001000Andersonetal,1991IncidenceRateper100,000FemalesMalesCancerandVTE