DIC弥漫性血管内凝血-北京协和医院血液科VIP免费

DIC的诊断与治疗北京协和医院血液科第一页，共三十九页。DIC的定义•DisseminatedIntravascularCoagulation•DIC是一种发生于多种疾病或特殊病理状态下，人体凝血系统被激活而引起中小血管内弥漫性微血栓形成及继发性纤溶亢进的综合征。•由于DIC开展过程中出现不同程度的血小板和凝血因子水平消耗性减少，也称之为“消耗性凝血病〞或“消耗性血栓出血性疾病〞。第二页，共三十九页。ClinicalconditionsassociatedwithDIC1.Sepsis/Severeinfection-----------44.6%2.Malignancy-------------------------20.7%–Solidtumors6.9%，AL13.8%.OccurrenceinAPL37~65%.3.Obstetricalcalamities------------13.4%–Amnioticfluidembolism,Abruptioplacentae,Deadfetus4.Trauma/Surgery-----------------7.4%5.Severehepaticfailure------------7.4%6.Vascularabnormalities–Kasabach-Merrittsyndrome，Largevascularaneurysms7.Organdestruction(e.g.,severepancreatitis)8.Severetoxicorimmunologicreactions–Snakebites，Recreationaldrugs，Transfusionreactions，Transplantrejection第三页，共三十九页。Mortality•DIC----DeathIsComing.•Mortalityrangesfrom31~86%,whetherornotheparinwasadministrated.•CorrelatedFactors:–Underlyingdisorders–Theextentoforgondysfuction–Thedegreeofhemostaticfailure–Increasingage第四页，共三十九页。ContactFactorPathwayTissueFactorPathway(IntrinsicPathway)(ExtrinsicPathway)KallikreinPrekallikrein“TissueDamage”XIIXIIaTissueFactorHKC-1InhibitorXIXIaIXCa++Ca++VIIIVIIIaIIaIIIIaVaVProteinCAPCProteinSPCInhibitorTMSKIIaIIaFibrinogenFibrinMonomerAT-IIIHCF-IIFibrinPolymerFibrinClotFDPXIIIaCa++Plasmin2AntiplasminPlasminogenUKt-PAPAI-1FibrinolysisSystemCoagulationandFibrinolysis(VIIa)XaIXaCa++PLXaXVIIaVIITFPICa++PLXIIIIIaCa++Ca++PLCa++PL第五页，共三十九页。TheSimplifiedMechanismofDIC第六页，共三十九页。DIC的失调控•Sepsis、Cancer、Trauma、Obstetricalcomplications:TF•LiverDisease:AT-III、PC/PS•Sepsis:TM、PC•Pregnancy:PS•APL、AmnioticFluidEmbolism、ProstateCancer:Plasmin第七页，共三十九页。ThrombinExplosionunderPathologicalConditions第八页，共三十九页。IXa(+VIII)Xa(+V)TF+VIIaThrombinFibrinogenFibrinDecreaseofAT-IIIImpairmentofPCSystemInsufficientTFPICytokines(IL-6,etc.)PlasminogenPlasminFibrinFDPsPAPAI-1GenerationofThrombinMediatedbyTFImpairmentofAnticoagulationPathwaySuppressionofFibrinolysisbyPAI-1FormationofFibrinInadequateRemovalofFibrinThrombosisofSmallandMidsizeVesselsPathogeneticPathwaysInvolvedinDIC第九页，共三十九页。AbnormalCoagulationinDIC第十页，共三十九页。PhysiologicAnticoagulantPathways第十一页，共三十九页。DysfunctionofthePCSysteminDIC第十二页，共三十九页。SchistocytesIntravascularFibrin第十三页，共三十九页。DIC临床表现频率•临床表现各异，根据6组报道平均发生率(WilliamsHematology-6thEdition，Table126-2)–1.出血表现：77.3%–2.肾损害：46.4%–3.呼吸道表现：42.2%–4.肝损害：39.5%–5.休克：34.5%–6.CNS表现：22.8%–7.血栓栓塞：22.2%–8.肢端苍白：6.8%–9.其它第十四页，共三十九页。DIC的实验室检查•MarkersofThrombinGeneration–D-dimer–3Ptest–Fibrinmonomer–FibrinopeptideA–Prothrombinfragment1+2–TAT•Screeningassaysforfactorsandplateletconsumption–PT–APTT–TT–Fibrinogen–Plateletcount•Ancillarytests–FDP–ELT–AT-III–FactorV/VIII–2-Antiplasmin第十五页，共三十九页。DIC的诊断标准•根据1994年武汉全国出血与血栓学术讨论会拟订以下标准：–1.临床表现–2.实验室指标第十六页，共三十九页。临床表现1、存在易引起DIC的根底疾病。2、有以下两项以上的临床表现多发性出血倾向。不易用原发病解释的微循环衰竭或休克。多发性微血...