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发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”。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，TransplantrejectionMortality•DIC----DeathIsComing.•Mortalityrangesfrom31~86%,whetherornotheparinwasadministrated.•CorrelatedFactors:–Underlyingdisorders–Theextentoforgondysfuction–Thedegreeofhemostaticfailure–IncreasingageContactFactorPathwayTissueFactorPathway(IntrinsicPathway)(ExtrinsicPathway)KallikreinPrekallikrein“TissueDamage”XIIXIIaTissueFactorHKC-1InhibitorXIXIaIXCa++Ca++VIIIVIIIaIIaIIIIaVaVProteinCAPCProteinSPCInhibitorTMSKIIaIIaFibrinogenFibrinMonomerAT-IIIHCF-IIFibrinPolymerFibrinClotFDPXIIIaCa++Plasmin2AntiplasminPlasminogenUKt-PAPAI-1FibrinolysisSystemCoagulationandFibrinolysis(VIIa)XaIXaCa++PLXaXVIIaVIITFPICa++PLXIIIIIaCa++Ca++PLCa++PLTheSimplifiedMechanismofDICDIC的失调控•Sepsis、Cancer、Trauma、Obstetricalcomplications:TF•LiverDisease:AT-III、PC/PS•Sepsis:TM、PC•Pregnancy:PS•APL、AmnioticFluidEmbolism、ProstateCancer:PlasminThrombinExplosionunderPathologicalConditionsIXa(+VIII)Xa(+V)TF+VIIaThrombinFibrinogenFibrinDecreaseofAT-IIIImpairmentofPCSystemInsufficientTFPICytokines(IL-6,etc.)PlasminogenPlasminFibrinFDPsPAPAI-1GenerationofThrombinMediatedbyTFImpairmentofAnticoagulationPathwaySuppressionofFibrinolysisbyPAI-1FormationofFibrinInadequateRemovalofFibrinThrombosisofSmallandMidsizeVesselsPathogeneticPathwaysInvolvedinDICAbnormalCoagulationinDICPhysiologicAnticoagulantPathwaysDysfunctionofthePCSysteminDICSchistocytesIntravascularFibrinDIC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-AntiplasminDIC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.实验室指标临床表现1、存在易引起DIC的基础疾病。2、有下列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。–多发性微血管栓塞的症状、体征，如皮肤、皮下、粘膜栓塞坏死及早期出现的肾、肺、脑等脏器功能不全。–抗凝治疗有效。实验室主要标准-同时有以下三项以上异常1.Plt.<100109/L或进行性下降（肝病、白血病血小板<50109/L）或有2项以上血小板活化产物升高（-TG,PF4,TXB2，GMP-140）。2.血浆Fibrinogen含量<1.5g/L（白血病及其他恶性肿瘤<1.8g/L，肝病<1.0g/L）...