醫(yī)學精品課件：（參考翻譯）05.2aplastic anemia-Li Wang

AplasticAnemia

(再生障礙性貧血)WangliHaematologyDepartmentTheFirstAffiliatedHospitalReviewWhatisthefunctionofbonemarrow骨髓Hematopoiesis造血Redbloodcells---transportoxygenandnutrients

Whitebloodcells---fightinfection

platelets—helpbloodclot凝結Bonemarrowhaematopoiesisfailure,HSC造血干細胞injuredPeripheralbloodpancytopenia全血細胞減少Typicalsymptoms:anemia,infection,bleedingDirectandindirectpathophysiologicpathwayscausingA.AThemaincharactersofAplasticAnemiaGeneralIntroductionClassification

ⅠⅡSevereModerateAbroadAcuteChronicHomeEpidemiologyIncidence

China

Annualincidence

7.4per100,000

6.01.4DistributionEpidemiologyfemalemaleInfant70’sChemicalfactors:drug

(anticarcinogen抗癌藥,chloramphenicol氯霉素,sulfonamide磺胺,NSAIDsetc)

chemicals

(benzene苯,insecticides殺蟲劑etc)Physicalfactors

radiation,XrayBiologicalfactors

viruses,severebacterium,infectionOtherpathogenicfactorsUnknownEtiologyPathogenesisFailureinhematopoieticstemorprogenitor祖cellsDefectinmicroenvironmentofhaematopoiesisImmuno-mediated免疫介導的

bonemarrowfailureGeneticaspects:HLAassociatedsusceptibility敏感SeedsSoilHarmfulInsectsPresentingsymptomsAcuteAAChronicAAOnsetacute,shorthistoryslowlyonset,longerhistoryAnemiasevere,remarkable顯著的relativelymoderateBleedingheavybleeding,Multiorgan多器官Hemorrhage出血moderate,skinandmucusInfectionmorefrequent,moderate,upperrespiratoryinfectionSepsis敗血癥Laboratoryfindings

Bloodcount（x109/L）

granulocytes<0.5>0.5

platelets<20>20

reticulocytes<15>15

BMmorphology骨髓形態(tài)學AcuteAAChronicAAmultiplacehypocellularitylackofmegakaryocyteslackofE,Gincreaseinmarrowfatincreasednon-HCSimilarfindings,focalareaactivehaematopoiesisLaboratoryfindings

Bloodcount（x109/L）

粒細胞<0.5>0.5

血小板<20>20

網(wǎng)織紅細胞<15>15

BMmorphology骨髓形態(tài)學AcuteAAChronicAA多種細胞減少巨核細胞缺乏紅細胞、粒細胞缺乏

骨髓脂肪增多非造血細胞增多相似表現(xiàn),核心區(qū)域造血活躍Bonemarrowaspiration吸NormalBMbiopsy

Thismarrowistakenfromamiddleagedperson,soitisabout50%cellular,withsteatocytes平衡細胞（？）admixed混合withthemarrowelements.NormalBM(highpowermagnification放大)

Notethepresenceofmegakaryocytes,erythroidislands,andgranulocyticprecursors粒細胞前體細胞.Thismarrowistakenfromtheposterioriliaccrest髂后上嵴inamiddleagedperson,soitisabout50%cellular,withsteatocytesadmixedwiththemarrowelements.AplasticanemiabiopsyHematopoieticelementsinthisbonemarrowbiopsyaremarkedly明顯的reduced.Ofcourse,RBC,plateletsandgranulocyteswilloftenbediminished減少的.MarrowFatAplasticanemia(HP)BMmorphologyofAcuteAARecoverphase恢復階段DiagnosticstandardofAA(1987)

DiagnosisDiagnosisSignBMPeriBlood外周血Exclusion排除DiagnosticstandardofAA(1987)

Decreasedperipheralbloodcount，absolutenumber絕對數(shù)量ofreticulocytesreduced

Withoutmegalosplenia脾腫大

DiagnosisBMmorphology

DiagnosisModerateorseverehypocellularity細胞缺乏foundatleastoneaspiration(biopsy)site.Nonhaematopoieticcells非造血細胞increaseinBM.Otherdiseaseswhichmaycausedecreasedbloodcountarecarefullyexcluded，suchasmyelodysplasticsyndrome骨髓增生異常綜合癥(MDS),acutemyelogenousleukemia急性骨髓性白血病(AML).

Routinetherapiesforanemiafailtowork.DiagnosisMyelodysplasticsyndrome--dysmorphic異形的featuresParoxysmalnocturnalhaemoglobinuria陣發(fā)性睡眠性血紅蛋白尿(PNH)HypoplasticAcuteLeukemia低增生性急性白血病OthersDifferentialDiagnosis–Pancytopenia全血細胞減少Anacquiredhaematopoieticstemcelldefectwithpredominant主要的haemolyticanaemia溶血性貧血.Adescriptiveterm描述詞fortheclinicalmanifestationofhaemolysis溶血andhaemoglobinuria血紅蛋白尿manifestbydarkcolouredurineinthemorning.PNH--ParoxysmalnocturnalhaemoglobinuriaParoxysmalnocturnalhaemoglobinuria.Historically歷史上的testwasHam’stest;showedredcelllysis溶解bycomplement補體activationinacidified酸化的serum.Currentlytestforabsentproteinsoncellsurface.CD55andCD59TreatmentSupportiveCareGrowthHormonesImmuneSuppressiveTherapyHematopoieticStemCellTransplantationRedcelltransfusionPlatelettransfusionManagementofneutropenia中性粒細胞減少privateroomfacemaskhandwashbroad-spectrumantibiotics廣譜抗生素iffeverSupportiveCareIndicatedforpatients>50yearsPatientswithnoHLAmatchedsiblingdonors同胞捐助者Anti-ThymocyteGlobulin抗胸腺細胞球蛋白(ATG)oranti-lymphocyteglobulin抗人淋巴細胞球蛋白(ALG),cyclosporin,methylprednisoneBestresultsareforcombinationtherapy.Responseisslow,4-12weekstoseeearlyimprovementImmunosuppressivetherapyResponserates60-70%Relapses復發(fā)arecommonandcontinuedsupportivecareneeded.Upto50%ofrelapsedpatientswillrespondto2ndcourseofimmunosuppressivetherapyAge<55years.Conditioning調(diào)節(jié)withCyclophosphamide環(huán)磷酰胺&ant