血小板聚集功能.ppt

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1、血小板的止血作用及检测王学锋上海交通大学医学院附属瑞金医院12直径2-4um,厚0.2-0.4um3星形，圆形，椭圆形，不规则形4图19人血涂片示聚集的血小板和嗜酸性粒细胞56Scanningelectionmicrographofnormalhumanbloodcells(E红细胞G粒细胞L淋巴细胞M单核细胞P血小板)7血液涂片中的血小板8TheNormalIn-SituProcessFIG1:NormalbloodvesselIntima/mediawithendothelialcellbasalme

2、mbranesmoothmusclecellscollagenfiberserythrocytethrombocytelumenleukocyteHemostasis9FIG2InjuredbloodvesselExposureofcollagenAdhesionofplateletsmediatedbyvWFActivationandde-granulationofplateletsAggregationofplateletserythrocytenon-activatedthrombocytelume

3、nendothelialcellbasalmembraneactivatedcollagen-boundthrombocytecollagenfiberssmoothmusclecellsHemostasisTheNormalIn-SituProcess10FIG3Plateletplugstabilizationbyfibrinlumenendothelialcellbasalmembraneerythrocytesactivatedthrombocytesplugsealingandstabiliz

4、ationbyfibrinformationTheNormalIn-SituProcessHemostasis11一、血小板的结构12图22血小板电镜像(1横切面2纵切面3膜4糖衣5线粒体6血小板颗粒7小管系8糖原颗粒)131415电镜下血小板超微结构：表面结构，骨架系统，细胞器，特殊膜系统静止相机能相16表面结构17表面结构细胞外衣糖蛋白的糖链（血小板膜受体的所在位置）细胞膜蛋白质与脂质开放管道系统的开口1819FibronectinThrombinTSPvWFCollagenFibrinogenvWF，

5、FibronectinGPIc/IIaGPVGPIVGPIa/IIaGPIb/IXGPIIb/IIIa1.糖蛋白：受体特性图3.血小板的表面结构模式图2ICSHCD42b/42aCD41/61CD36CD49b/2920d-granulea-granuleGPIb-IXreceptor-complexvonWillebrand-FactorfibrinogenGPIIb-IIIareceptor-complexReleaseofglycoproteinsvWFfibrinogencoagulationfac

6、torsReleaseofADPserotonincalciumetcendothelialcellplateletPrimaryHemostasisNormalPlateletFunctioncollagenfibers21d-granulea-granuleGlanzmannThrombasteniaBernard-SoulierSyndromeplateletNoreleasefromgranules:Graysyndrome(noa-granules)StoragePoolDisease(nod-g

7、ranules)Secretiondefects(granulesarepresentbutsecretionisinhibited)PrimaryHemostasisCongenitalPlateletDysfunctions22VonWillebrandDiseaseMechanismPrimaryHemostasisd-granulea-granuleGPIb/Ixreceptor-complexvonWillebrand-JürgensSyndromeplateletcollagenfibers23

8、重要糖蛋白缺乏所导致的疾病?24生化组成磷脂酰胆碱（PC）磷脂酰乙醇胺（PE）膜磷脂鞘磷脂＋甘油磷脂磷脂酰丝氨酸（PS）磷脂酰肌醇（PI）溶血卵磷脂胆固醇糖脂膜脂质75%~80%20%~25%2%~5%2.膜脂质SPH25Phosphatidylcholine，PCPhosphatidylethano-lamine，PEPhosphatidylserine，PSPhosphatidylinositol，PISph