凝血和弥散性血管内凝血PPT课件.ppt

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1、REGULATIONOFCOAGULATION/DISSEMINATEDINTRAVASCULARCOAGULATIONREGULATIONOFCOAGULATIONIntroductionCoagulationnecessaryformaintenanceofvascularintegrityEnoughfibrinogentoclotallvesselsWhatcontrolsclottingprocess?COAGULATIONCASCADEVa/Xa/PLVIIIa/IXa/PLorVIIa/TFFXIIFXIIaTFFVIIFGFHMWK

2、FXIFXIaVIIa/TForFVIIaCa+2FIXFIXaCa+2Ca+2Ca+2VIIIVIIIaTVVaTCa+2FXFXaCa+2Ca+2PTTCommonPathwayMiddleComponentsSurfaceActiveComponentsINTRINSICPATHWAYEXTRINSICPATHWAYCOAGULATIONINHIBITORSTissueFactorPathwayInhibitor(TFPI)LipoproteinAssociatedCoagulationInhibitor(LACI)ExtrinsicPat

3、hwayInhibitor(EPI)ComplexeswithFactorsVIIa/TF/Xa;inactivatesXaAntithrombinIII/HeparinCofactorII/HeparinBindsandInactivatesEnzymesProteinC/ProteinS/ThrombomodulinCleaves&InactivatesCofactors(Va&VIIIa)Plasminogen-3ºhemostasisCleavesFibrinATIII+ThrombinATIII.ThrombinHeparinANTITHR

4、OMBINIII-MECHANISMOFACTIONPROTEINCACTIVATIONProteinC+ThrombinActivatedProteinCTTTMProteinCActivatedProteinCThrombinThrombomodulin+**CaPROTEINC-MECHANISMOFACTIONAPCFactorVa/FactorVIIIaProSPLiFVa/FVIIIaCOAGULATIONINHIBITORSTissueFactorPathwayInhibitor(TFPI)LipoproteinAssociatedC

5、oagulationInhibitor(LACI)ExtrinsicPathwayInhibitor(EPI)ComplexeswithFactorsVIIa/TF/Xa;inactivatesXaAntithrombinIII/HeparinCofactorII/HeparinBindsandInactivatesEnzymesProteinC/ProteinS/ThrombomodulinCleaves&InactivatesCofactors(Va&VIIIa)Plasminogen-3ºhemostasisCleavesFibrinANTI

6、COAGULANTPROTEINDEFICIENCYDiseaseentitiesHeterozygousProteinDeficiencyIncreasedVenousThrombosisOccasionalIncreasedArterialThrombosisWarfarinInducedSkinNecrosisHomozygousProteinDeficiencyNeonatalPurpuraFulminansFibrinogenolysisChronicDICANTICOAGULANTPROTEINDEFICIENCYDominantIncr

7、easedVenousThrombosisYoungAgeofThrombosisNoPredisposingFactorstoThrombosisIncreasedThrombinGenerationPositiveFamilyHistoryRecessiveNohistoryofthrombosisNofamilyhistoryNeonatalPurpuraFulminansIncreasedThrombinGenerationACTIVATEDPROTEINCRESISTANCE1stdescribedbyDahlback,1994Hallma

8、rk:FailureofactivatedProteinCtoprolongaPTTFirstnotedin