弥散性血管内凝血.ppt

弥散性血管内凝血Chapter11,中山医学院病理生理教研室邓宇斌,DIC一、DIC原因和发病机制二、促进DIC发生发展的因素（诱发困素）三、DIC的分期和分型四、DIC的功能代谢变化（病理生理变化）五、DIC防治的病理生理基础,第一节概述,1.血液的凝固与抗凝流动性血液运输载体方向性内（）凝血系统凝血外（）血小板：粘聚释,凝抗凝栓塞失衡凝抗凝出血倾向2.DIC的概念出血病因微血栓后休克致凝继发纤溶亢进果栓塞溶血120种病：感染、肿瘤、产科意外,Introduction,DICischaracterizedbytheactivationofthecoagulationsystemwithresultantconsumptionofavarietyofcoagulationproteinsandplatelets,whichresultsinhemorrhagicdiathesisandischemicinjurytovarioustissues.,1.BloodCoagulationItispropagatedbyanenzymaticeventstermedcoagulationcascade.ContactfactorsandtheintrinsicpathwayTissuefactorandextrinsicpathway,2.FibrinolysisItistheresultoftheactionofplasmin,aproteolyticenzymeproducedfromaninertplasmaprecursor(plasminogen)bytheactionofvarioussubstancestermedplasminogenactivators.,HumoralplasminogenactivatorsTissueplasminogenactivatorsFibrinorfibrinogendegradationproductsFDP(Significantbiologicalactivity)FragmentsX,YandE(potentantithrombins)FragmentsYandD(inhibitfibrinpolymerization),aaaPCAPCTM+灭活PS(+)C4bC4bPS(-),酶纤溶FDP酶ATPC抗APCTM+a凝PSPGI2VECTM,第二节DIC的病因发病学,一、发病原因及机理1.VEC广泛受损原因感染炎症、免疫损伤（抗磷脂综合征）高低温、放射损伤缺血缺氧酸中毒,EtiologyofDIC,1.acuteDIC(1)septicemia(2)severetrauma(3)obstetricaccidents(4)shock2.subacuteDIC(1)malignanttumors(2)retaineddeadfetus3.chronicDIC(1)gianthemangioma(2)systemiclupuserythematosus(SLE),机理胶原暴露凝VEC释放受损合成PGI2TXA2抗凝表达TMAPC,2.血细胞大量受损RBC受损感染：疟疾原因：溶血G6PDase：蚕豆病免疫损伤：异型输血红细胞素（）机理：释ADPP聚集,WBC激活或受损坏死白血病细胞释原因化疗受损机理炎症激活合成、释（内毒素、补体、LC、P、AgAb）,P激活或受损原发性：免疫损伤（抗P抗体抗磷脂抗体）继发性：DIC粘（GPb胶原）聚（GPbafg）TXA2等P聚、血管收缩机理PF111提供“反应面”aCa2+aaCa2+aPF3PF3,3.大量致凝物质入血肿瘤细胞坏死（包括产科意外）组织细胞带负电颗粒物质（内毒素）a胰蛋白酶其它丝氨酸蛋白水解酶a蝰蛇毒,PathogenesisofDIC,1.extensivedamageofvascularendothelialcellsIntrinsicclottingcascade2.severetissueinjuryExtrinsicclottingreaction,3.excessivedestructionofthecirculatingbloodcellsGenerationofprocoagulant-activesubstancesIntravascularcoagulation4.otherthromboplasticmaterialsenteringthebloodActivationofclottingsystemthroughthecontactofbloodwithanabnormalsurface,theneteffectsaresummarizedasfollows:1.lossofplasmafibrinogenasitisconsumedbytheclottingprocessandbytheactionofplasma.2.lossofotherclottingfactorsnotably,and,astheyareusedupduringtheoperationoftheclottingcascade.3.fallintheplateletcount,astheplateletsaggregateandleavethecirculation.4.appearanceoffibrindegradationproducts,asplasminactsonitssubstrates.,二、诱因与发生机理消除致凝物质功能血液凝血活性抗凝活性1.单核吞噬细胞系统功能内毒素血症、糖皮质激素、脾消除功能：致凝物、a、凝纤产物,2.肝功能严重障碍灭活活化凝血因子合成AT、PC枯否细胞吞噬功能3.血液的高凝状态凝血活性凝血物质：怀孕、肿瘤、应激抗纤溶：胎盘、药抗凝活性抗肝素：HAT、PC、TM等4.血流郁滞,PredisposingfactorstoDIC,1.impairmentoftheclearancemechanism.2.hypercoagulablestate.3.disorderofmicrocirculation.,第三节DIC的分期及分型,高凝期分期消耗性低凝期继发性纤溶亢进期急性按发病速度亚急性分慢性型代偿型按代偿情况失代偿型过度代偿型,TypesofDIC1.acuteDICMultisidebleedingdiathesisThromboticcomplicationsusuallySeverebleedingleadtoshockandsevereischemicchangeinorgans2.subacuteDICRarelybleedingTheevidenceofDICcanbedetectedbylaboratoryexaminations3.chronicDIC,StageofDIC,1.hypercoagulablestage2.hypocoagulablestage3.secondaryfibrinolyticstage,第四节临床表现,1.出血凝血物质消耗性酶：破坏凝血因子继发性纤溶亢进aFDP抗凝：竞争性抑制aP聚血管壁受损及溶栓,ConsequencesofDIC,1.disturbanceofcoagulation-bleeding(1)theconsumptionofclottingfactorsandplatelets(2)theactivationoffibrinolyticsystem(3)theproductionoffibrindegradationproducts(FDPs),2.休克出血回心血量微血栓阻断通路CO心泵功能:心肌DICBP右心后负荷:肺DIC外周阻力:四个酶系统激活A、B肽扩血管物质FDP(通透性)激肽C3a、C5a,2.disturbanceofcirculation-shockMicrothromobusincapillariesandvenulesBloodreturningdecreaseCardiacmuscledamageCardiacoutputandbloodvolumereduceEffectivecirculatingbloodvolumedecreaseHypotension,3.栓塞微血栓器官功能BP供血障碍4.溶血：微血管病性溶血性贫血,3.ischemictissuedamage-dysfunctionofmultipleorgansRenalinsufficiencyAcuteadrenalfailurePituitarynecrosisAdultoracuterespiratorydistresssyndrome(ARDS)Convulsionandcoma,4.microangiopathichemolyticanemia(MHA)characteristicmorphologicabnormalityoftheredbloodcellsTwistedcells,crenatedcells,triangularcells,helmet-shapedcells,andmicrospherocytesareseenonthebloodsmear.,Pathophysiologicalbasisoflaboratorydiagnosis,1.detectionofplateletcountanditsfunction2.determinationofclottingfactors3.determinationofactivityoffibrinolysis(1)thrombintimetest(TT)(2)plasmapro