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ID:19499072
大小:396.50 KB
页数:46页
时间:2018-10-02
《临床医学陈勤奋溶血性贫血课件》由会员上传分享,免费在线阅读,更多相关内容在教育资源-天天文库。
1、溶血性贫血HemolyticAnemia华山医院血液科陈勤奋HEMOLYTICANEMIA(reducedRBClifespan)AnemiaofincreaseddestructionNormochromic,normochromicanemiaShortenedRBCsurvivalReticulocytosis--ResponsetoincreasedRBCdestructionIncreasedindirectbilirubinIncreasedLDHMechanismsandCausesINTRACORPUSCULARHEMOLYSISMembraneAbnormalitiesM
2、etabolicAbnormalitiesHemoglobinopathiesEXTRACORPUSCULARHEMOLYSISNonimmuneImmuneMembraneDefectsMicroskeletaldefectsHereditaryspherocytosisIncreasedsensitivitytocomplementParoxysmalnocturnalhemoglobinuriaEnzymopathiesGlucose6-PhosphateDehydrogenaseDeficiencyPyruvateKinaseDeficiencyHemoglobinopathies
3、HemoglobinopathyThalassemiaExtracorpuscularHemolysisNonimmuneInfectiousChemicalThermalOsmoticExtracorpuscularHemolysisImmuneAllrequireantigen-antibodyreactionsTypesofreactionsdependenton:ClassofAntibodyNumber&SpacingofantigenicsitesoncellAvailabilityofcomplementEnvironmentalTemperatureFunctionalst
4、atusofreticuloendothelialsystemManifestationsIntravascularhemolysisExtravascularhemolysisExtracorpuscularHemolysisImmuneAntibodiescombinewithRBC,&eitherActivatecomplementcascade,&/orOpsonizeRBCforimmunesystemIf1,ifallofcomplementcascadeisfixedtoredcell,intravascularcelllysisoccursIf2,&/orifcomplem
5、entisonlypartiallyfixed,macrophagesrecognizeFcreceptorofIg&/orC3bofcomplement&phagocytizeRBC,causingextravascularRBCdestructionClassificationIntravascularExtravascularclinicalfeatureschronicpallor、jaundice、splenomegalycholelithiasisaplasticcrisisacutealgor、hyperpyrexia、sorewaisthemoglobinuriajaund
6、ice、anaemialaboratoryexaminationIncreasedRBCdestructionReducedRBClifespanIndirecthyperbilirubinemiaErythroidhyperplasiaReticulocytosisErythroblasts,anisopoikilocytosis,spherocytesinbloodsmearErythroidhyperplasiainbonemarrowdiagnosisanddifferentialdiagnosishemolysisornot?typeofhemolysisanotheranemi
7、a?anotherjaundicewithanemia?anotherjaundicewithoutanemia?TreatmentRemovethecausesImmunosupressivedrugWashedRBCtransfusionSplenectomySymptomatictreatmentHereditarySpherocytosisDefectiveorabsentspectrinmoleculeLead
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