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AplasticAnemia(再生障碍性贫血)WangliHaematologyDepartmentTheFirstAffiliatedHospitalReviewWhatisthefunctionofbonemarrowHematopoiesisRedbloodcells---transportoxygenandnutrientsWhitebloodcells---fightinfectionplatelets—helpbloodclotBonemarrowhaematopoiesisfailure,HSCinjuredPeripheralbloodpancytopeniaTypicalsymptoms:anemia,infection,bleedingDirectandindirectpathophsiologicpathwayscausingA.AThemaincharactersofAplasticAnemiaGeneralIntroductionClassificationⅠⅡSevereModerateAbroadAcuteChronicHomeEpidemiologyIncidenceChinaAnnualincidence7.4per100,000AcuteChronic6.01.4DistributionEpidemiologyfemalemaleInfant70’sChemicalfactors:drug(anticarcinogen,chloramphenicol,sulfonamide,NSAIDsetc)chemicals(benzene,insecticidesetc)Physicalfactorsradiation,XrayBiologicalfactorsviruses,severebacterium,infectionOtherpathogenicfactorsUnknownEtiologyPathogenesisFailureinhematopoieticstemorprogenitorcellsDefectinmicroenvironmentofhaematopoiesisImmuno-mediatedbonemarrowfailureGeneticaspects:HLAassociatedsusceptibilitySeedsSoilHarmfulInsectsPresentingsymptomsAcuteAAChronicAAOnsetacute,shorthistoryslowlyonset,longerhistoryAnemiasevere,remarkablerelativelymoderateBleedingheavybleeding,multiorganhemorrhagemoderate,skinandmucusInfectionmorefrequent,moderate,upperrespiratoryinfectionsepsisLaboratoryfindingsBloodcount（x109/L）granulocytes<0.5>0.5platelets<20>20reticulocytes<15>15BMmorphologyAcuteAAChronicAAmultiplacehypocellularitylackofmegakaryocyteslackofE,Gincreaseinmarrowfatincreasednon-HCSimilarfindings,focalareaactivehaematopoiesisBonemarrowaspirationNormalBMbiopsyThismarrowistakenfromamiddleagedperson,soitisabout50%cellular,withsteatocytesadmixedwiththemarrowelements.NormalBM(highpowermagnification)Notethepresenceofmegakaryocytes,erythroidislands,andgranulocyticprecursors.Thismarrowistakenfromtheposterioriliaccrestinamiddleagedperson,soitisabout50%cellular,withsteatocytesadmixedwiththemarrowelements.AplasticanemiabiopsyHematopoieticelementsinthisbonemarrowbiopsyaremarkedlyreduced.Ofcourse,RBC,plateletsandgranulocyteswilloftenbediminished.MarrowFatMarrowFatAplasticanemia(HP)BMmorphologyofAcuteAARecoverphaseDiagnosticstandardofAA(1987)DiagnosisDiagnosisSignBMPeriBloodexclusionDiagnosticstandardofAA(1987)Decreasedperipheralbloodcount，absolutenumberofreticulocytesreducedWithoutmegalospleniaDiagnosisBMmorphologyDiagnosisModerateorseverehypocellularityfoundatleastoneaspiration(biopsy)site.NonhaematopoieticcellsincreaseinBM.Otherdiseaseswhichmaycausedecreasedbloodcountarecarefullyexcluded，suchasmyelodysplasticsyndrome(MDS),acutemyelogenousleukemia(AML).Routinetherapiesforanemiafailtowork.DiagnosisMyelodysplasticsyndrome--dysmorphicfeaturesParoxysmalnocturnalhaemoglobinuria(PNH)HypoplasticAcuteLeukemiaOthersDifferentialDiagnosis--PancytopeniaAnacquiredhaematopoieticstemcelldefectwithpredominanthaemolyticanaemia.Adescriptivetermfortheclinicalmanifestationofhaemolysisandhaemoglobinuriamanifestbydarkcolouredurineinthemorning.PNH--ParoxysmalnocturnalhaemoglobinuriaParoxysmalnocturnalhaemoglobinuria.HistoricallytestwasHam’stest;showedredcelllysisbycomplementactivationinacidifiedserum.Currentlytestforabsentproteinsoncellsurface.CD55andCD59TreatmentSupportiveCareGrowthHormonesImmuneSuppressiveTherapyHematopoieticStemCellTransplantationRedcelltransfusionPlatelettransfusionManagementofneutropeniaprivateroomfacemaskhandwashbroad-spectrumantibioticsiffeverSupportiveCareIndicatedforpatients>50yearsPatientswithnoHLAmatchedsiblingdonorsAnti-ThymocyteGlobulin(ATG)oranti-lymphocyteglobulin(ALG),cyclosporin,methylprednisoneBestresultsareforcombinationtherapy.Responseisslow,4-12weekstoseeearlyimprovementImmunosuppressivetherapyResponserates60-70%Relapsesarecommonandcontinuedsupportivecareneeded.Upto50%ofrelapsedpatientswillrespondto2ndcourseofimmunosuppressivetherapyAge<55years.ConditioningwithCyclophosphamide&antithymocyteglobulin,withcyclosporinandmethotrexate.Longtermoverallsurvival=80-90%Chronicgraftversushostdisease(GVHD)remainsaproblemfor25-40%ofpatients.HSCT-HLAidenticalsiblingHSCT21yearmanPresentedwithfatigue,pale,tirednessHb11.0WBC2.6,neutrophils1.1,platelets45,MCV104.B12/folate/ferritinwerenormal.Mainphysicalexaminationwasunremarkable.CasePresentationMarrowaspiratehypocellularMarrowbiopsyhypocellularDiagnosis?Treatment?CasePresentationThankyouJohnRadcliffeHospital