弥散性血管内凝血DIC课件.ppt

*组织因子（tissuefactor,TF）是由263个aa组成的跨膜糖蛋白，正常情况下，血管外层的平滑肌细胞、成纤维细胞、周细胞、星形细胞、足状突细胞可恒定的表达TF，以备止血。而与血液接触的内皮细胞、单核细胞、嗜中性粒细胞及巨噬细胞正常时不表达TF。但在各种感染或炎症介质刺激下，这些细胞可在短时间内诱导出TF，引起凝血反应*微血管病性溶血性贫血（MicroangiopathicHemolyticAnemia）:慢性DIC和有些亚急性DIC常出现hemolyticanemia的症状，外周血涂片可发现新月形、盔甲形、星形、三角形等变形红细胞，被称为裂体细胞（schistocyte）。在DIC发病过程中，fibrin除形成microthrombus外，也有相互交织联结在微血管中形成纤维蛋白丝、纤维蛋白网，特别在DIC早期小血管中这些纤维蛋白丝网较多。当红细胞随血流快速在微血管中流动时，被纤维蛋白丝网撞击、粘挂，或红细胞嵌在VEC较大裂隙处。血流冲击使这些红细胞受到挤压、切割等机械损伤，最终红细胞变形、破裂形成schistocyte。Schistocyte变形能力显著降低，脆性增高，在血流的冲击、碰撞下容易破裂，发生溶血。将这种因微血管发生病理变化而导致红细胞破裂引起的贫血，称为microangiopathichemolyticanemia。外周血涂片中发现较多schistocyte（超过红细胞数2%），虽非DIC所特有，却是诊断DIC的重要参考指标。Microangiopathichemolyticanemiascomprisethrombocytopenicthromboticpurpura,thehemolytic–uremicsyndrome,chemotherapy-inducedmicroangiopathichemolyticanemia,malignanthypertension,andtheHELLPsyndrome(hemolysis,elevatedliver-enzymelevels,andalowplateletcountoccurringinassociationwithpreeclampsia).20Althoughsomecharacteristicsofmicroangiopathichemolyticanemiasandtheresultingthromboticocclusionofsmallandmidsizevesselsmaymimictheclinicalpictureofdisseminatedintravascularcoagulation,thesedisordersareadistinctgroupofdiseases.Thecommonfeatureofthemicroangiopathichemolyticanemiasappearstobeendothelialdamage,whichcausestheadhesionandaggregationofplatelets,theformationofthrombin,andtheimpairmentoffibrinolysis.Inpatientswiththrombocytopenicthromboticpurpuraandthehemolytic–uremicsyndrome,theacquireddeficiencyofaproteasethatcleavesmultimersofvonWillebrandfactorleadstotheaccumulationoflargemultimersofthisfactor.21Acardinalsignofmicroangiopathichemolyticanemiaisthepresenceoffragmentedredcells(schistocytes)inthebloodsmear.Althoughhemolyticanemiaandschistocytesarealsosometimespresentinpatientswithseveredisseminatedintravascularcoagulation(becauseofthepresenceofintravascularfibr