陈勤奋－溶血性贫血.ppt

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1、溶血性贫血HemolyticAnemia华山医院血液科陈勤奋HEMOLYTICANEMIA(reducedRBClifespan)AnemiaofincreaseddestructionNormochromic,normochromicanemiaShortenedRBCsurvivalReticulocytosis--ResponsetoincreasedRBCdestructionIncreasedindirectbilirubinIncreasedLDHMechanismsandCausesINTRACORPUSCULARHEMOLY

2、SISMembraneAbnormalitiesMetabolicAbnormalitiesHemoglobinopathiesEXTRACORPUSCULARHEMOLYSISNonimmuneImmuneMembraneDefectsMicroskeletaldefectsHereditaryspherocytosisIncreasedsensitivitytocomplementParoxysmalnocturnalhemoglobinuriaEnzymopathiesGlucose6-PhosphateDehydrogenaseDe

3、ficiencyPyruvateKinaseDeficiencyHemoglobinopathiesHemoglobinopathyThalassemiaExtracorpuscularHemolysisNonimmuneInfectiousChemicalThermalOsmoticExtracorpuscularHemolysisImmuneAllrequireantigen-antibodyreactionsTypesofreactionsdependenton:ClassofAntibodyNumber&Spacingofantig

4、enicsitesoncellAvailabilityofcomplementEnvironmentalTemperatureFunctionalstatusofreticuloendothelialsystemManifestationsIntravascularhemolysisExtravascularhemolysisExtracorpuscularHemolysisImmuneAntibodiescombinewithRBC,&eitherActivatecomplementcascade,&/orOpsonizeRBCforim

5、munesystemIf1,ifallofcomplementcascadeisfixedtoredcell,intravascularcelllysisoccursIf2,&/orifcomplementisonlypartiallyfixed,macrophagesrecognizeFcreceptorofIg&/orC3bofcomplement&phagocytizeRBC,causingextravascularRBCdestructionClassificationIntravascularExtravascularclinic

6、alfeatureschronicpallor、jaundice、splenomegalycholelithiasisaplasticcrisisacutealgor、hyperpyrexia、sorewaisthemoglobinuriajaundice、anaemialaboratoryexaminationIncreasedRBCdestructionReducedRBClifespanIndirecthyperbilirubinemiaErythroidhyperplasiaReticulocytosisErythroblasts,

7、anisopoikilocytosis,spherocytesinbloodsmearErythroidhyperplasiainbonemarrowdiagnosisanddifferentialdiagnosishemolysisornot?typeofhemolysisanotheranemia?anotherjaundicewithanemia?anotherjaundicewithoutanemia?TreatmentRemovethecausesImmunosupressivedrugWashedRBCtransfusionSp

8、lenectomySymptomatictreatmentHereditarySpherocytosisDefectiveorabsentspectrinmoleculeLead