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DISORDERSOFHEMOSTASIS Dept ofHematologyZhongShanHospital FuDanUniversityProf JlAN MINXU GeneralConsiderations Disordersofhemostasisdefectsinplateletnumberorfunctionproblemsinfibrinclot coagulation Bleedingduetoplateletdisordersistypicallymucosalordermatologicincludeepistaxis gumbleeding menorrhagia gastrointestinalbleeding purpura andpetechiae GeneralConsiderations Petechiaeareseenalmostexclusivelyinconditionsofthrombocytopeniaandnotplateletdysfunction Bleedingduetocoagulopathymayoccurasdeepmusclehematomasaswellasskinbleeding Spontaneoushemarthrosesareseenonlyinseverehemophilia GeneralConsiderations Definition disorderofhemostasisspontaneousbleedingabnormalbleedingfollowingtraumaMachanism vesselwalldefectthrombocytopenia plateletdisfunctioncoagulationdisorder Normalhemostasisandcoagulation hemostasis 1 Vesselwall 1 vesselspasm reflexspasmS HT slowdownbloodflow 2 subendothelialconnectivetissue encouragingplateletadhesion activation aggregation 3 releasingTF activatingF triggeringintrinsicandextrinsicpathway 4 pressionofhaematoma Normalhemostasisandcoagulation 2 platelet 1 adhesion tosubendothelialconnectivetissuebyGP b vWF 2 aggregation formationofplateletthrombusbyGP b a 3 release phosphatide ArachidonicAcid TXA2furtherplateletactivationandadhesionviolentvesselspasm Normalhemostasisandcoagulation coagulation 1 Rearrangementofplateletmembranephosphatidate PF3 2 Tissueinjury TFrelease 3 Subendothelialconnectivetissue atriggeringintrinsicandextrinsicpathway whitethrombus redthrombus Normalhemostasisandcoagulation coagulation nature acascadeofenzymesactivationnotactivebeforeactivatedcomponent 12coagulationfactor 11inplasma tissuefactor protein excludeCa codewith etc PKHMWK Normalhemostasisandcoagulation 3 course 1 thromboplatinintrinsic a a a Ca PF3extrinsic III VII Ca a Ca PF3 2 thrombin prothrombinthromboplatin Cathrombin Boneuetal SangThromboseVaisseaux 1998 10 291 313 II VIIa 细胞的促凝血酶原激酶 Ca2 Xa XIa 内源性系统 外源性系统 X IX XIIIa XI XII XIIa 溶解性纤维蛋白 纤维蛋白原 纤维蛋白 凝块 VII 凝血酶IIa IXa VIIIa Ca2 PL Xa因子的核心作用 Normalhemostasisandcoagulation 3 fibrin fibrinogenthrombinfibrinmonomer polymerXIIIapolymer 4 functionofthrombin coagulation activate strengthen platelet aggregation releasefibrinolysis activateplasminogen Normalhemostasisandcoagulation Anticoagulantsystem 1 anticoagulin 1 eachcoagulatefactorandtheircomplexhasitsowninhibitor 2 important AT 70 anticoagulantactivityserineproteinaseinhibitor a a a a DK Normalhemostasisandcoagulation 3 proteinCsystem PCTMAPC PSsterilizeVa VIIIa InhibitPAI PhysiologyandPathology APCI a trypsinize TM thrombinactivatorsterilize a aPCAPCreleaseplasminogenCa fibrolysisCa PSmodulatetheactivationofc accelerationinhibitionAPC ActivatedProteinC Normalhemostasisandcoagulation fibrinolysis plasminogenplasminogenactivatorplasmin PAI a2 AP 2 fibrinolysis fibrinogen fragmentX fragmentY fragmentE FDP polypeptideABCfragmentDfragmentD Abnormality 1 Vesselwall heredity Telangiectasisaquired deficiencyofVitC VitPhypersensitivity anaphylactoidpurpuraelse Abnormality 2 Platelet 1 thrombocytopenia production AA leukemia infection drugdestruction ITPexhaust TTP HUS DIC Abnormality 2 thrombocythemia primary Primaryhemorrhagicthrombocythemiasecondary CML splenectomy infectiontrauma 3 functiondeficiency heredity thrombastheniasecondary drug renalfailureliverdisease Abnormality 3 Coagulation 1 heredity hemophilia willabranddisease 2 aquired liverdisease renalfailure VitKdeficiency DIC 3 anticoagulantagents Ab drug Diagnosis Historyandphysicalexam PE 1 history cause sex hereditybackgroundcourse2 PE petechialbleeding organ muscle jointcavity Diagnosis Labexamination 1 filter vessel platelet BT plt CRTcoagulation CT APTT PT TT Diagnosis 2 particular 1 platelet morphology bulk scatter gatheringadhesion aggregation inducer Adr ADP collagen ristocetin thrombasthenia thrombusdisease Diagnosis 2 Coagulation APTT CT VIII IX XIPTCT V VII X IITT fibrinogen anticoagulantagentsCoagulantfactortest quantityactivation PreventionandTherapy 1 Prevention drug aspirin persantin indomethacindisease liverdisease renalfailureheredity preventtraumer operation PreventionandTherapy 2 hemostasis 1 supplyment coagulantfactor freshplasma blood platelet 2 drug vessel VitC VitP Carbazochrome Predplatelet Etamsylate Predcoagulation VitK prothrombincomplexfibrinolysis EACA PAMBA TranexamicAcid 3 local oppression decreasemovement 窈鹁谞鋚敎呗跦囼詊弓実键鱤摄允杰術儽鞂皀坁愹浥呯瞋铗攈抴膟辪轱鰥鹹缢唆桘畝綞鷬洽楽宕儴俎廜爔迲峚綃丝案杮烛佅阆另厯篩卽施樂庽龞嬱鶭郼鑛蜵愂蕄菲犝辿豂懿炂鮼殩米桊隮雀弛鷧熄鲈墳匐集褝蝁傍巒熛鰟榫晉昚垹阥郘砋歘衅馏荢邌鷽甌巄鏋奆灒毌纈滻瀵惖萔猘讫亽徖洣琄荠庮霚覻薵隞趁碊抶罾綼甒皋跄绲卦唧帎僺鯞呺睸癋菣鷠些莅爧吂艔戳燪伩摐慞勜舗度斠肵分騟琮成魑尴嵠齧底塂炑搫號鸝媋營歹蛱叜訂糛亾禹湺蔂蛀賭匙郕喪梁溪劼铴賄亭菾倎荂笖蛎尠螑媧鏄冺斺儡禫掇囮澼宓檪婩肪蹣丒鞨碘闞縶蹼奟熛魞霆窲昦毞靎伎裰锉敿浶悚觊逮詂莔慡屁挞督蜂乁淌砍裲儕忘甡褲繄埊榿蜘批芞睞悎釛菪熼謒紬誼琙叭肜郱闶娻蓿削抖咭鼿鹼仃淏綨驄搳礭臘胣馏莁懬咔鮕艏歃赘龚榛鸞仸悌嗹脂簣毭祴伊紋势禃僋虥烪禮魃犹風傲醏駺檃橓擨舃攖舊涁艍簀尃韚鴽宆鉻覸 11111111144487看看 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