出血性疾病概述刘霆教授一定义由于血管壁异常、血小板数量质量改变、凝血和抗凝血功能障碍而表现为自发出血或轻微外伤后出血不止或过多的临床症候群凝血与抗凝机制的病理生理基础Primary--ISecondary--IIBloodvessels& PlateletsCoagulationfactors& Fibrinolyticagents凝血与抗凝机制的病理生理基础Vascularaspect:血管收缩-血流缓慢内皮细胞表达并释放vwF-血小板粘附聚血管损伤-胶原暴露-内源性凝血组织因子释放-外源性凝血释放t-PA-纤维蛋白溶解系统表达及释放TM—PC凝血与抗凝机制的病理生理基础Plateletaspect:Function:保持血管内皮完整性adhesion:需有-胶元、vwF、GPIbaggregation:(诱聚剂)GPⅡb/Ⅲa-变形-白色血栓secretion(a颗粒、致密颗粒):有5-HT、TXA2、ADP;Ca2+、PF3血管收缩、血小板聚集、凝血凝血与抗凝机制的病理生理基础vWFvWFvWFPlateletAdherencecollagenGpIbvWFGpIbvWFGpIbvWF凝血与抗凝机制的病理生理基础GpIbvWFvWFvWFBernard-Soulier Syndrome-巨大血小板综合症vonWillebrand'sDisease凝血与抗凝机制的病理生理基础GpIbvWFDenseGranuleIIIaIIbIIIaIIbADPADPIIIaIIbPlateletAggregationFibrinogenEDDD凝血与抗凝机制的病理生理基础DenseGranuleAlpha-granuleLysosomeADPß-thromboglobulinPlateletfactor4Platelet-derivedGrowth FactorFibrinogenFactorVHydrolasePlateletReleaseFunction凝血与抗凝机制的病理生理基础
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