內(nèi)科學(xué) 出血性疾病(英文)

HemorrhagicDisorders

1ContentSummaryCausesandMechanismofDiseaseClinicalFeaturesInvestigationofLabCriterionofDiagnosisManagement2DefinitionWhenthenormalhemostaticmechanismfails,majorhemorrhagemayfollowminortraumaormayariseapparentlyspontaneously.3Mechanismof

normalhemostasisVascularmechanismPlateletmechanismBloodcoagulationmechanism4Normalhemostasis567redthrombus8AnticoagulationandfibrinolysticmechanismAnticoagulationsystemAT-ⅢProteinCsystem:PC,PS,TMTFPIHeparin910AnticoagulationandfibrinolysticmechanismFibrinolysissystemPlasminogenTissue-typeplasminogenactivator(t-PA)Urokinase-typeplasminogenactivator(u-PA)Inhibitors:α2-plasmininhibitor(α2-PI),plasminogenactivatorinhibitor-1(PAI-1)11AnticoagulationandfibrinolysticmechanismPlasminogenActivator

InhibitorKallekrein (intrinsic) t-PA,u-PA PAI (extrinsic) α2-PI plasminfibrinogen(fibrin) FDP1213ClassificationvasculardisordersplateletdisordersdisordersofbloodcoagulationdisordersofanticoagulationsystemandfibrinolyticsystemComplexdisordersofhemostaticmechanism14Classification:VasculardisordersHereditaryorcongenital:Hereditaryhemorrhagictelangiectasia（遺傳性出血性毛細(xì)血管擴(kuò)張癥）,hereditarydisordersofconnectivetissuesuchasEhlers-Danlossyndrome.Secondary:infections,chemicalfactorsordrugs,disordersofmetabolism(VitaminCorPdeficiency),pathologicalchangesofvascularwall(atherosclerosis),connectivetissuediseases.Allergy:allergicpurpura.Otherpurpuras:purpurasimplex,senilepurpura,mechanicpurpura,paraproteinemia.15Classification:

plateletdisordersThrombocytosisThrombocytopathyThrombocytopenia16Classification:plateletdisorders17Classification:plateletdisordersThrombocytosisPrimary:essentialthrombocythemiaSecondary:infections,injury,post-splenectomy,chronicmyelocyticleukemia,othermyeloproliferativedisorders(suchaspolycythemiavera真性紅細(xì)胞增多癥）)18Classification:plateletdisordersFunctionalabnormalitiesofplateletsHereditary:giantplateletsyndrome(Bernard-Souliersyndrome),

thrombasthenia(血小板無力癥）,Acquired:duetodrugs,uremia,liverdiseases,dysproteinemias.19Classification:

disordersofbloodcoagulationHereditaryorcongenital:hemophiliaA(FⅧdeficiency),hemophiliaB(FⅨdeficiency),factorⅪdeficiency(formerlyhemophiliaC),hypothrombinogenemia,hypofibrinogenemia,vonWillebrand’sdisease,othercoagulationfactorsdeficiency,includingdeficiencyofactivated-proteinCinhibitorstructuralabnormalities.Acquired:VitaminKdeficiency,severeliverdiseases,disseminatedintravascularcoagulation(DIC)etc.

20Classification:

disordersofanticoagulationsystemandfibrinolyticsystemdisordersofanticoagulationsystemandfibrinolyticsystemoverdoseofheparinordicumarol,liberationoftissueplasminogenactivatorintothecirculation,antibodiesagainstFⅧ,Ⅸ,thromboplastin,thrombin,heparinoidanticoagulant,DIC.21Classification:

Complexdisorders

ofhemostaticmechanismHereditaryorcongenitalvWDAcquiredDICLiverdisease22Etiologicaldiagnosis:HistoryWhoAgeSexFamilyhistoryWhenPredisposingcauseDrugingestion?NutritionstatusanyconcomitantdiseasesFrequencyofthebleedingevent23Etiologicaldiagnosis:

Symptomsandsigns

Where:Thesiteofbleeding(skin,mucousmembrane,GItract,solidorgan,jointormuscle?)What:Thephysicalcharacteristicsofthebleeding(petechial瘀點(diǎn)?Purpura紫癜?Ecchymoses瘀斑?hematoma?)OtherconcomitantsymptomsandsignsVitalsigns:HR,RR,BP,etc.2425

hemophilia26Differentialdiagnosis

Table.Theclinicaldistinctionbetweendisordersofvesselsandplateletsanddisordersofbloodcoagulation

27Etiologicaldiagnosis:

Laboratoryexaminations

ScreeningtestsFortheVasculardisordersandplateletdisorderscapillaryfrangibilitytestbleedingtime(BT)plateletcountclotretractiontest(CRT)28Etiologicaldiagnosis:

Laboratoryexaminations

Forthedisordersofbloodcoagulationactivatedpartialthromboplastintime(APTT)prothrombintime(onestagemethod)(PT)thrombintime(TT)clottingtime(CT)293031323334Etiologicaldiagnosis:

LaboratoryexaminationsConfirmatorytestsForvasculardisordersvWFEndothelin-1(ET-1)TM35Etiologicaldiagnosis:

Laboratoryexaminations

ConfirmatorytestsForplateletdisordersmegakaryocytesinmarrowmorphologyofplateletsplateletadhesiontestaggregationtestinducedbyADP,adrenalinecollagen,arachidonicacidanalysisofplateletglycoproteinsetc.36Etiologicaldiagnosis:

Laboratoryexaminations

ConfirmatorytestsFordisordersofbloodcoagulationmeasurementofdifferentfactorantigensandcoagulantactivities37Etiologicaldiagnosis:

Laboratoryexaminations

ConfirmatorytestsFordisordersofanticoagulationAT-Ⅲantigenandactivity,TATPCFactorⅧ:CantibodyLupus-typeanticoagulant,cuorin-typeanticoagulant38Etiologicaldiagnosis:

Laboratoryexaminations

ConfirmatorytestsFordisordersoffibrinolysis3PtestFDPD-dimerPlasminogent-PA39Etiologicaldiagnosis:

LaboratoryexaminationsSpecialtestsForhereditarydisordersorsomespecialdisordersProteinstructuralanalysisAminoacidsequencingGeneanalysisimmunopathology40Etiologicaldiagnosis:

diagnosticprocedure

hemorrhagicdisorder?classification(vessel?BPC?Coagulation?)Whichfactor?quantityorquality?Congenital?Hereditary?Acquired?Geneandmolecularbiologyanalysis41TreatmentEtiologicaltreatmentandpreventionAcquireddiseasesDrugsthatimpairplateletfunctionsshouldbeavoided.Vigoroustreatmentoftheunderlyingdiseases,suchasliverdisease.42TreatmentEtiologicaltreatmentandpreventionHereditarydiseasesNoradicaltreatment.Avoidintramuscularinjections,surgicaloperationandtrauma.Plansurgicalprocedurescarefully,iftheoperationisinevitable.Antenataldiagnosis43TreatmentTreatmentaccordingtopathophysiologyDrugsdecreasingthecapillaryfragility:Rutin,VitaminC,adrenosem.Drugsused