血小板聚集功能PPT培训课件VIP免费

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23直径2-4um,厚0.2-0.4um4星形，圆形，椭圆形，不规则形5图图1919人血涂片示聚集的血小板和嗜酸性粒细胞人血涂片示聚集的血小板和嗜酸性粒细胞67ScanningelectionmicrographofnormalhumanbloodcellsScanningelectionmicrographofnormalhumanbloodcells(E(E红细胞红细胞GG粒细胞粒细胞LL淋巴细胞淋巴细胞MM单核细胞单核细胞PP血小板血小板))8血液涂片中的血小板9TheNormalIn-SituProcessFIG1:NormalbloodvesselIntima/mediawithendothelialcellbasalmembranesmoothmusclecellscollagenfiberserythrocytethrombocytelumenleukocyteHemostasis10FIG2•Injuredbloodvessel•Exposureofcollagen•AdhesionofplateletsmediatedbyvWF•Activationandde-granulationofplatelets•Aggregationofplateletserythrocytenon-activatedthrombocytelumenendothelialcellbasalmembraneactivatedcollagen-boundthrombocytecollagenfiberssmoothmusclecellsHemostasisTheNormalIn-SituProcess11FIG3PlateletplugstabilizationbyfibrinlumenendothelialcellbasalmembraneerythrocytesactivatedthrombocytesplugsealingandstabilizationbyfibrinformationTheNormalIn-SituProcessHemostasis12一、血小板的结构13图图2222血小板电镜像血小板电镜像(1(1横切面横切面22纵切面纵切面33膜膜44糖衣糖衣55线粒体线粒体66血小板颗粒血小板颗粒77小管系小管系88糖原颗糖原颗粒粒))141516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17表面结构18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19FibronectinThrombinTSPvWFCollagenFibrinogenvWF，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2ICSHICSHCD42b/42aCD41/61CD36CD49b/2921-granule-granuleGPIb-IXreceptor-complexvonWillebrand-FactorfibrinogenGPIIb-IIIareceptor-complexReleaseof•glycoproteins•vWF•fibrinogen•coagulationfactorsReleaseof•ADP•serotonin•calcium•etcendothelialcellplateletPrimaryHemostasisNormalPlateletFunctioncollagenfibers22-granule-granuleGlanzmannThrombasteniaBernard-SoulierSyndromeplateletNoreleasefromgranules:•Graysyndrome(nogranules)•StoragePoolDisease(nogranules)•Secretiondefects(granulesarepresentbutsecretionisinhibited)PrimaryHemostasisCongenitalPlateletDysfunctions23VonWillebrandDiseaseMechanismPrimaryHemostasis-granule-granuleGPIb/Ixreceptor-complexvonWillebrand-JürgensSyndromeplateletcollagenfibers24重要糖蛋白缺乏所导致的疾病?25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SPHPhosphatidylcholine，PCPhosphatidylethano-lamine，PEPhosphatidylserine，PSPhosphatidylinositol，PISphingomyelin，SPH溶血磷脂plateletfactor32.膜脂质PF3★图4.血小板的表面结构模式图3（二）血小板骨架系统和收缩蛋白（二）血小板骨架系统和收缩蛋白①.微管Microtubule②.微丝Microfilaments③.膜下细丝Submembranefilaments图5.血小板的骨架系统模式图4①①②②③③28AandB二聚体细丝微管微管维持血小板形态29骨架系统和收缩蛋白•微丝肌动蛋白细丝及肌球蛋白粗丝,参与血小板收缩活动，伪足形成和释放膜下细丝（三）血小板（三）血小板细胞器和内容物细胞器和内容物DDD1.α-1.α-颗粒（颗粒（granulegranule）：）：P-selectinP-selectin，，PF4PF4，，β-β-TGTG，，TSPTSP，，FnFnaaaa2.致密颗粒（densebody，δ颗粒）：ADPADP，，ATPATP3.溶酶体颗粒（γ颗粒）：组织蛋白酶组织蛋白酶DD、、EE、、OO等等图6.血小板细胞器模式图531细胞器和内容物致密颗粒颗粒...