出血性疾病總論

OverviewofBleedingDiseaseDefinition

Bleedingdisorderreferstoagroupofdiseases,thatnormalhemostaticmechanismdisordercausedspontaneousbleeding,orexcessivebleedingafterminorinjury

systemsinvolvedinhemostasisVascularSpasm:thesmoothmuscleinbloodvesselwallscontractsimmediately,whiletheotherhemostaticmechanismsbecomeactive.PlateletPlugFormation:thekeystagesofthisprocessare:plateletadhesion,plateletreleasereaction,andplateletaggregationBloodCoagulation-coagulationsystem,anticoagulationsystemfibrinolyticsystemtheroleofvascularsysteminhemostasismechanismofvasoconstrictiontheendotheliumcellseventsfollowingvascularinjury

Stimulateplateletadhesion-Whitethrombusformation

Activationofcoagulationsystem-solidredthrombusformation血管在止血中的作用血管損傷血管收縮兒茶酚胺/5-HT/血管緊張素/加壓素/內皮素/TXA2血流減弱血管內皮表達粘附分子配體內皮下成分暴露血小板粘附、聚集、釋放GPIb/vWFGPIIb-IIIa膠原/纖原ADP/TXA2/

-TGPAF/Fg/vWF/Ca++合成、表達TFThrombin形成Fibrin形成血栓形成激活外源凝血系統Vesselwallabnormalities

(vascularpurpura)CongenitalvascularwallabnormalitiesHereditarytelangiectasiaAcquiredthevesselwallabnormalities

immunity

allergicpurpura

non-immune

Infection:bacteria,viruses,fungi,etc.

Chemical:drugs,chemicaltoxins,etc.

Metabolic:vitaminCdeficiency,long-termusehormone

Mechanical:trauma,pressure,etcSchematicdiagramofplateletmorphologytheroleofplateletsinhemostasisplateletadherencetocollagen:GpIbPlateletaggregation：GpIIb/IIIacomplexplateletreleasereaction:ADP,PAF,TXA2formationofplateletthrombosisandstabilizationoftheclot血小板在止血中的作用血管損傷膠原暴露血小板粘附血小板聚集膠原VWF血小板GPIb-IX纖維蛋白原GPIIb-IIIa血小板異常引起的出血舉例血小板減少血小板粘附異常GPIb缺乏：巨大血小板病vWF缺乏：血管性血友病血小板聚集異常GPIIb-IIIa缺乏：血小板無力癥纖維蛋白原缺乏：血小板聚集障礙VWFGPIb-IX纖維蛋白原GPIIb-IIIaQuantitativeandQualitative

PlateletDisordersQuantitativeDisordersofPlateletsThrombocytopeniaRelatedtoDecreasedProduction,AlteredDistribution,ImmuneEffect,Consumption.ThrombocytosisInheritedQualitativeDisordersofPlateletsDisordersofAdhesionPlateletReleaseDefectsAcquiredDefectsofPlateletFunctionVascularDisordersLeadingtoPlateletDysfunctionInvivocoagulationcascadeInvitrocoagulationcascadeCoagulationfactordisordersHereditaryCoagulationDisorders

HemophiliaA(VIIIfactordeficiency)HemophiliaB(IXfactordeficiency)Acquiredcoagulationfactorabnormalities

Coagulationfactordisorders

—AcquiredDecreasedsynthesisSevereliverdisease:factorVIII!VitaminKantagonists:II,VII,IX,XIncreasedconsumptionanddestructionFibrinolyticsystemBleedingdisorders

clinicalmanifestationsAgeGenderBleedingincentivesDrugs,Surgery,trauma,Spontaneousbleeding,

Infectionsetc.Bleedingfrequencytransient,recurrentorpersistentFamilyHistory

CHARACTERISTIC?PATTERNS?OF?BLEEDING?IN?SYSTEMIC?DISORDERS?OF?HEMOSTASIS皮膚出血點（2mm以內）皮膚紫癜(3

5mm)

口腔粘膜出血點皮膚大片淤斑關節血腫出血性疾病：實驗室檢查

—反映血管壁功能的試驗

常規試驗

意義 毛細血管脆性試驗血管脆性、通透性 毛細血管鏡 毛細血管襻形態、數量、結構，血流狀態

病理活檢 血管及周圍組織的結構 出血性疾病：實驗室檢查

—反映血小板數量和功能的檢查

常規試驗 意義

血小板計數血小板數量血涂片血小板形態血小板聚集聚集功能血小板粘附粘附功能毛細血管脆性試驗血小板數量和功能，小血管結構功能，凝血出血時間（出血時間測定器法）同上出血性疾病：實驗室檢查

—反映凝血因子含量和功能的試驗

常規試驗 意義

活化的部分凝血時間(APTT)內源性凝血功能凝血酶原時間(PT)外源性凝血功能凝血酶時間(TT)纖維蛋白原質或量、抗凝物質有無纖維蛋白原FDP,D-Dimer纖維蛋白原、纖維蛋白原降解產物

凝血因子抗原和活性各凝血因子含量和功能Bleedingdisorders:

diagnosticprocedures

Bleedingdisorder？Causeofdisease？

Vascular?platelet?Coagulationdisorder?multiplefactor?Labteststoconfirmthediagnosis病例男性，50歲,臨床表現：血尿、黑便、皮膚大片瘀斑數天，一月前類似發作史。自訴無誘因。既往無異常出血史，無類似家族史。無特殊藥物服用史。體格檢查：輕度貧血貌，無慢性肝病面容，下肢大片瘀斑。

實驗室檢查會有哪些發現？病例

—實驗室檢查CBC：wbc11.37×109/L,Hb77g/L,Bpc287×109/L肝腎功能：正常PT、APTT明顯延長，TT正常，纖維蛋白原正常

？病例

—維生素K依賴性凝血因子缺乏吸收不良綜合