溶血性贫血英文版

溶血性贫血

HemolyticAnemia华山医院血液科陈勤奋5/9/20241溶血性贫血英文版HEMOLYTICANEMIA

(reducedRBClifespan)

AnemiaofincreaseddestructionNormochromic,normochromicanemiaShortenedRBCsurvivalReticulocytosis--ResponsetoincreasedRBCdestructionIncreasedindirectbilirubinIncreasedLDH5/9/20242溶血性贫血英文版MechanismsandCausesINTRACORPUSCULARHEMOLYSISMembraneAbnormalitiesMetabolicAbnormalitiesHemoglobinopathiesEXTRACORPUSCULARHEMOLYSISNonimmuneImmune5/9/20243溶血性贫血英文版MembraneDefectsMicroskeletaldefectsHereditaryspherocytosisIncreasedsensitivitytocomplementParoxysmalnocturnalhemoglobinuria5/9/20244溶血性贫血英文版EnzymopathiesGlucose6-PhosphateDehydrogenaseDeficiencyPyruvateKinaseDeficiency5/9/20245溶血性贫血英文版HemoglobinopathiesHemoglobinopathyThalassemia5/9/20246溶血性贫血英文版ExtracorpuscularHemolysis

NonimmuneInfectiousChemicalThermalOsmotic5/9/20247溶血性贫血英文版ExtracorpuscularHemolysis

ImmuneAllrequireantigen-antibodyreactionsTypesofreactionsdependenton:ClassofAntibodyNumber&SpacingofantigenicsitesoncellAvailabilityofcomplementEnvironmentalTemperatureFunctionalstatusofreticuloendothelialsystemManifestationsIntravascularhemolysisExtravascularhemolysis5/9/20248溶血性贫血英文版ExtracorpuscularHemolysis

ImmuneAntibodiescombinewithRBC,&eitherActivatecomplementcascade,&/orOpsonizeRBCforimmunesystemIf1,ifallofcomplementcascadeisfixedtoredcell,intravascularcelllysisoccursIf2,&/orifcomplementisonlypartiallyfixed,macrophagesrecognizeFcreceptorofIg&/orC3bofcomplement&phagocytizeRBC,causingextravascularRBCdestruction5/9/20249溶血性贫血英文版ClassificationIntravascularExtravascular5/9/202410溶血性贫血英文版clinicalfeatureschronicpallor、jaundice、splenomegalycholelithiasisaplasticcrisisacutealgor、hyperpyrexia、sorewaisthemoglobinuriajaundice、anaemia5/9/202411溶血性贫血英文版laboratoryexaminationIncreasedRBCdestructionReducedRBClifespanIndirecthyperbilirubinemiaErythroidhyperplasiaReticulocytosisErythroblasts,anisopoikilocytosis,spherocytesinbloodsmearErythroidhyperplasiainbonemarrow5/9/202412溶血性贫血英文版5/9/202413溶血性贫血英文版diagnosisanddifferentialdiagnosishemolysisornot?typeofhemolysisanotheranemia?anotherjaundicewithanemia?anotherjaundicewithoutanemia?5/9/202414溶血性贫血英文版TreatmentRemovethecausesImmunosupressivedrugWashedRBCtransfusionSplenectomySymptomatictreatment5/9/202415溶血性贫血英文版HereditarySpherocytosisDefectiveorabsentspectrinmoleculeLeadstolossofRBCmembrane,leadingtospherocytosisDecreaseddeformabilityofcellIncreasedosmoticfragilityExtravascularhemolysisinspleen5/9/202416溶血性贫血英文版HereditarySpherocytosisPathophysiology--RBCmembraneproteindefects(spectrindeficiency)resultingcytoskeletoninstabilityFamillyhistoryClinicalfeatures—splenomegaly5/9/202417溶血性贫血英文版HereditarySpherocytosisLaboratoryfeatures

-hemolyticanemia

-bloodsmear-microspherocytes

-abnormalosmoticfragilitytest

-positiveautohemolysistest

-preventionofincreasedautohemolysisbyincluding

glucosein

incubationmediumTreatment--splenectomy5/9/202418溶血性贫血英文版HereditarySpherocytosis5/9/202419溶血性贫血英文版HereditarySpherocytosis

OsmoticFragility5/9/202420溶血性贫血英文版ParoxysmalNocturnalHemoglobinuria(PNH)PNHisanacquiredchronichemolyticanemiawhicharisesfromasomaticmutationinahematopoieticstemcell.Mosthematopoiticcelllinesmaybeaffectedbytheintrinsicmembranedefect.Thisdefectrenderstheredcellshighlysusceptibletocomplementmediatedlysisresultinginthecharacteristichemolysis.5/9/202421溶血性贫血英文版ParoxysmalNocturnalHemoglobinuria(PNH)Pathogenesis

-anacquiredclonaldisease,arisingfromasomaticmutationinasingleabnormalstemcell

-glycosyl-phosphatidyl-inositol(GPI)anchorabnormality

-deficiencyoftheGPIanchoredmembraneproteins(CD55andCD59)

-redcellsaremoresensitivetothelyticeffectofcomplement

-intravascularhemolysis

5/9/202422溶血性贫血英文版urinealiquotofPNH5/9/202423溶血性贫血英文版clinicalmanifestationpancytopeniapassageofdarkbrownurineinthemorningvenousthrombosis(Budd-ChiariSyndrome)5/9/202424溶血性贫血英文版LaboratoryfeaturesPancytopeniaChronicurinaryironlossSerumironconcentrationdecreasedHemoglobinuriaHemosiderinuriaPositiveHam’stest(acidhemolysistest),sugar-watertestSpecificimmunophenotypeofbloodcells(CD59,CD55)

5/9/202425溶血性贫血英文版AttentionAA-PNHsyndromeAA→PNHPNH→AAPNHwithAAAAwithPNH5/9/202426溶血性贫血英文版TreatmentavoidcauseswashedRBCtransfusionirontherapyallogenicbonemarrowtransplantation5/9/202427溶血性贫血英文版G-6-PDDeficiencyacutehemolyticanemiacongenitalnonspherocytichemolyticanemianeonatalhyperbilirubinemia（kernicterus）favism5/9/202428溶血性贫血英文版Levelneededforprotectionvsordinaryoxidativestress5/9/202429溶血性贫血英文版AutoimmuneHemolysisDuetoformationofautoantibodiesthatattackpatient’sownRBC’sTypecharacterizedbyabilityofautoantibodiestofixcomplement&siteofRBCdestructionOftenassociatedwitheitherlymphoproliferativediseaseorcollagenvasculardisease5/9/202430溶血性贫血英文版AutoimmuneHemolyticAnemiawarm-reactiveantibodiesprimarysecondarycold-reactiveantibodiescoldagglutininsyndromeparoxysmalcoldhemoglubinuria5/9/202431溶血性贫血英文版Autoimmunehematolysis

WarmTypeIgG+C3IgGC35/9/202432溶血性贫血英文版Autoimmunehematolysis

WarmTypeUsuallyIgGantibodiesFixcomplementonlytolevelofC3,ifatallImmunoglobulinbindingoccursatalltempsFcreceptors/C3brecognizedbymacrophagesHemolysisprimarilyextravascular70%associatedwithotherillnessesResponsivetosteroids/splenectomy5/9/202433溶血性贫血英文版Clinicalmanifestationanemia、jaundice、splenohepatomegaliaITP+AIHA=Evenssyndrome5/9/202434溶血性贫血英文版LaboratoryexaminationBlood：anemia；Ret；erythroblasts,anisopoikilocytosisBonemarrow：erythroidhyperplasia

CoombsTest5/9/202435溶血性贫血英文版CoombsTest-DirectLooksforimmunoglobulin&/orcomplementofsurfaceofredbloodcell(normallyneitherfoundonRBCsurface)Coombsreagent-combinationofanti-humanimmunoglobulin&anti-humancomplementMixedwithpatient’sredcells;ifimmunoglobulinorcomplementareonsurface,CoombsreagentwilllinkcellstogetherandcauseagglutinationofRBCs5/9/202436溶血性贫血英文版CoombsTest-IndirectLooksforanti-redbloodcellantibodiesinthepatient’sserum,usingapanelofredcellswithknownsurfaceantigensCombinepatient’sserumwithcellsfromapanelofRBC’swithknownantigensAddCoombs’reagenttothismixtureIfanti-RBCantigensareinserum,agglutinationoccurs5/9/202437溶血性贫血英文版Diagnosis