溶血性贫血 课件.ppt

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1、溶血性贫血HemolyticAnemiaHEMOLYTICANEMIA(reducedRBClifespan)AnemiaofincreaseddestructionNormochromic,normochromicanemiaShortenedRBCsurvivalReticulocytosis--ResponsetoincreasedRBCdestructionIncreasedindirectbilirubinIncreasedLDHMechanismsandCausesINTRACORPUSCULARHEMOLYSISMembraneAbnor

2、malitiesMetabolicAbnormalitiesHemoglobinopathiesEXTRACORPUSCULARHEMOLYSISNonimmuneImmuneMembraneDefectsMicroskeletaldefectsHereditaryspherocytosisIncreasedsensitivitytocomplementParoxysmalnocturnalhemoglobinuriaEnzymopathiesGlucose6-PhosphateDehydrogenaseDeficiencyPyruvateKinaseD

3、eficiencyHemoglobinopathiesHemoglobinopathyThalassemiaExtracorpuscularHemolysisNonimmuneInfectiousChemicalThermalOsmoticExtracorpuscularHemolysisImmuneAllrequireantigen-antibodyreactionsTypesofreactionsdependenton:ClassofAntibodyNumber&SpacingofantigenicsitesoncellAvailabilityofc

4、omplementEnvironmentalTemperatureFunctionalstatusofreticuloendothelialsystemManifestationsIntravascularhemolysisExtravascularhemolysisExtracorpuscularHemolysisImmuneAntibodiescombinewithRBC,&eitherActivatecomplementcascade,&/orOpsonizeRBCforimmunesystemIf1,ifallofcomplementcascad

5、eisfixedtoredcell,intravascularcelllysisoccursIf2,&/orifcomplementisonlypartiallyfixed,macrophagesrecognizeFcreceptorofIg&/orC3bofcomplement&phagocytizeRBC,causingextravascularRBCdestructionClassificationIntravascularExtravascularclinicalfeatureschronicpallor、jaundice、splenomegal

6、ycholelithiasisaplasticcrisisacutealgor、hyperpyrexia、sorewaisthemoglobinuriajaundice、anaemialaboratoryexaminationIncreasedRBCdestructionReducedRBClifespanIndirecthyperbilirubinemiaErythroidhyperplasiaReticulocytosisErythroblasts,anisopoikilocytosis,spherocytesinbloodsmearErythroi

7、dhyperplasiainbonemarrowdiagnosisanddifferentialdiagnosishemolysisornot?typeofhemolysisanotheranemia?anotherjaundicewithanemia?anotherjaundicewithoutanemia?TreatmentRemovethecausesImmunosupressivedrugWashedRBCtransfusionSplenectomySymptomatictreatmentHereditarySpherocytosisDefect

8、iveorabsentspectrinmoleculeLeadstolossof