彌散性血管內凝血英文版

Disseminated

IntravascularCoagulation(DIC)

TheriskfactorsandetiologyofDICThepathogenesisofDICThepathophysiologyofDICTheclinicalfeaturesofDICThediagnosisofDICThetherapyofDICCONTENTSWhatIsDIC?It’sconsideredan“acquiredbleedingdisorder”It’snotadiseaseentitybutaneventthatcanaccompanyvariousdiseaseprocessesIt’saParadoxicalClinicalPresentation“clottingandhemorrhage”TheDefinitionAnacquiredsyndromecharacterizedbytheintravascularactivationofcoagulationwithlossoflocalization

arisingfromanycausedamagetothemicrovasculatureifsufficientlysevere,itcanproduceorgandysfunctionUnderlyingConditionsAssociatedwithDICBasicdiseaseratioofthediseasetoall(%)Infectiondiseases36.94Obstetriccomplications24.81Malignancies24.21Surgeryandtrauma4.34Iatrogenicfactor1.45Otherfactors8.25WhatIsTheCausesOfDIC?DICandInfectiousDiseaseSeveresepsisisthemostcommonclinicalconditionassociatedwithDICBacterialinfectionoccursin30-50%ofGram-sepsisLipopolysaccharide(endotoxin)

Gram+sepsisexotoxin(e.g.staphylococcala-haemolysin)DICandseveretraumaEspeciallyseenafterbraintraumareleaseoffatandphospholipidCytokineactivationsimilarpatterntoseveresepsis“Systemicinflammatoryresponsesyndrome”aftertrauma50-70%associatedwithDICDICandCancerSolidtumoursmetastaticcancer10-15%Haematologicalcanceracuteleukaemia 15%‘Cancerpro-coagulant’tissuefactorAcutepromyelocyticleukaemiaDICandhyperfibrinolyticstateDICandObstetricalDisordersAbruptioplacentae,amnioticfluidembolism,fetaldeathinutero,septicabortionReleaseofthromboplastin-likematerialUsuallyshort-livedandself-limitingPre-eclampsiaDICandGiantHaemangiomaLocalactivationofcoagulationsystemsystemicdepletionoflocallyconsumedclottingfactorsandplateletsActivatedcoagulationfactorsreachsystemiccirculationDICGianthaemangioma25%Largeaorticaneurysm0.5-1%TheriskfactorsandetiologyofDICThepathogenesisofDICThepathophysiologyofDICTheclinicalfeaturesofDICThediagnosisofDICThetherapyofDICCONTENTSHowIsTheDICHappen?PathologicPathwaysExtrinsic(endothelial)ShockortraumaInfections(grampositiveandgramnegativesepsis,aspergillosis)Obstetriccomplications(eclampsia,placentaabruptio,fetaldeathsyndrome)Malignancies:APL,AML,cancersofthelung,colon,breast,prostateIntrinsic(bloodvessel)Infectiousvasculitis(certainviralinfections,rockymountainspottedfever)VasculardisordersIntravascularhemolysis(hemolytictransfusionreactions)Miscellaneous:snakebite,pancreatitis,liverdiseaseDICischaracterizedbytheincreasinglossoflocalizationorcompensatedcontrolincoagulationactivation.PathogenesisofDICIncreasedthrombingenerationDepressionofphysiologicanticoagulationmechanismDelayedremovaloffibrinduetoimpairedfibrinolysisActivationofThrombinistheFocusinDICactivationofprothrombininhibitingATactivationof

Plateletactivationoffibrinolysisactivationof

Ⅹ、Ⅻ、ⅩⅢactivationofVthrombinTheriskfactorsandetiologyofDICThepathogenesisofDICThepathophysiologyofDICTheclinicalfeaturesofDICThediagnosisofDICThetherapyofDICCONTENTSFormationofMicrothrombusDisfunctionofCoagulationDisturbanceofMicrocirculationTheriskfactorsandetiologyofDICThepathogenesisofDICThepathophysiologyofDICTheclinicalfeaturesofDICThediagnosisofDICThetherapyofDICCONTENTSClinicalFeaturesOnsetmaybeAcuteorChronicAcuteDICDevelopsrapidlyoveraperiodofhoursPresentswithsuddenbleedingfrommultiplesitesTreatedasamedicalemergencyChronicDICDevelopsoveraperiodofmonthsMaybesubclinicalEventuallyevolvesintoanacuteDICpatternSignsandSymptoms

MostcommonsignofDICisbleeding-manifestedbyecchymosis,petechiae,andpurpura -bleedingfrommultiplesiteseitheroozingorfrankbleeding -coolandormottledextremitiesmaybenoted -dyspneaandchestpainifpleuraandpericardiuminvolvement -hematuriaTHEFUNCTIONOFORGANSFAILURE

MicrothrombusLocalnecrosis&ulcermayappearMicroangiopathichaemolyticanaemiaPeripheralbloodpictureAnaemiaThrombocytopeniaFragmentedredcells(schistocytes)AfeaturecommontoseveralconditionsDICThromboticthrombocytopenicpurpuraHaemolyticUraemicSyndromeMainFeaturesofDICFeaturesAffectedPatients(%)Bleeding64Renaldysfunction25Hepaticdysfunction19Respiratorydysfunction16Shock14Centralnervoussystemdysfunction2TheriskfactorsandetiologyofDICThepathogenesisofDICThepathophysiologyofDICTheclinicalfeaturesofDICThediagnosisofDICThetherapyofDICCONTENTSHowToDiagnoseDIC?DiagnosisofDICClinicalsettingLaboratorytestsCriteriaUnderlyingdiseaseknowntobeassociatedInitialplateletcount<100×109/L,orrapiddeclineinBPCProlongationofclottingtimes(PT&APTT)PresenceoffibrindegradationproductsLowlevelsofcoagulationinhibitors(e.g.antithrombin)Lowfibrinogenlevelinseverecasesconsumptivecoagulopathy

SecondaryfibrinolysisProthrombintime（PT）Activated

PTT（APTT）Plateletcount（PLT）

Fibrinogen（Fbg）Antithrombin（AT）FactorⅧ：C

Fibrindegradationproduct(FDP)D-dimer3Ptest

Solublefibrinmonomer（SF）

Laboratoryfindingsin

DICDIC:Phases

OvertDIC

Decompensatedform

Non-overtDIC

MoresubtlehemostaticdysfunctionTheInternationalSocietyofThrombosisandHaemostasis(ISTH)TheJapaneseMinistryofHealthandWelfare(JMHW)

TheJapaneseAssociationforAcuteMedicine(JAAM)DifferentialDiagnosisSeriousHepatitishepaticfunction

(jaundice,FVIII,D-Dimer)Primaryhyperfibrinolysis

onlyfactorⅠdecreaseThromboticThrombocytopenicPurpura(TTP)

vWF-cleavingprotease(vWF-cp)

MechanismofVWF-CPinpathogenesisofTTPUL-VWFbloodvesselVWF-CPnormalVWFmultimersbloodvesselVWF-CPdeficiencyTTPMicro-thrombusformationTheriskfactorsandetiologyofDICThepathogenesisofDICThepathophysiologyofDICTheclinicalfeaturesofDICThediagnosisofDICThetherapyofDICCONTENTSHowToTreatDIC?TreatmentofDIC

Treatmentofunderlying

disorder

Anticoagulants

Supplyclottingfactors

&platelets

OthersManagementofDICTreatmentofunderlyingdisorder

veryimportantIdentifyunderlyingcauseandtreatAllothertherapiesaretemporizingAnticoagulantslowdoseheparinlowmolecularweightheparinnewthrombininhibitors(ATIIIindependent)usefulforclinicallyovertthromboembolismorextensivedepositionoffibrinTheprincipleofheparin/LMWHtherapyEarlierperiodofDIC（hypercoagulation）Platelets&factorsdecrease，microthrombusAfteraddingcoagulationfactors&pltinconsumptionhypocoagulationphaseRefractorinessshockContraindication

①Activebleedingafteroperation&trauma②Seriousbleedingrecently③DICcausedbyvenene④Severitydefectofcoagulationfactors&obviouslyhyperfibrinolysisTheapplyingofanticoagulants

Heparin＜12500U/d，＜5000U/6h，IVorH，×3～5d

lowmolecularweightheparin(LMWH)

75～150IUAⅩ/kg.d，H，×3～5dUsingHeparinasanticoagulant,APTTmustbemonitored,thebestdoseofHeparinisthatwhentheAPTTisprolongedto1.5～2.0times.UsingLMWHdon’tneedtomonitorAPTTManagementofDICPlateletsandPlasmatotreatbleedingtendencytocoveraninvasiveprocedureforpatientswithahighriskofbleedingClottingfactorconcentratesovercomeslargevolumesofplasmabutnotadvocatedbecause:1)containssmallamountofactivatedfactors2)DICresultsindeficiencyofmultiplefactorsConcentratesofcoagulationinhibitorsAntithrombinconcentratereducessepsisrelatedmortalityimprovementofDICandorganfunctionSupportivetherapeuticoptioninsevereDICAntifibrinolyticagentsGenerallynotrecommendedfibrinolysisisalreadyimpairedinDICmayenhancefibrindepositionForbleedinginDICassociatedwithprimaryorsecondaryhyperfibrinolysise.g.acutepromyelocyticleukaemiaStratificationtherapyofDICConsumptivehypocoagulationperiod

AnticoagulantsSupplyclottingfactors

&plateletsDiffusedmicrothrombusperiodAnticoagulants

SecondaryhyperfibrinolysisSupplyclottingfactors

&plateletsAntifibrinolyticagentsEarlierstageIntermediatestageAdvancedstage

A26year-oldfemale.Shewasnotedtohavedisproportionateandasymmetricmacrodactylinherhandsandfeetseveralmonthsafterherbirth.Shewasgenerallyasymptomatic,hadnoeasybruisingorotherobviousmucocutaneousbleeding.Andtherewasnomentalretardationa