医学精品课件：（参考翻译）05.2aplastic anemia-Li Wang

AplasticAnemia

(再生障碍性贫血)WangliHaematologyDepartmentTheFirstAffiliatedHospitalReviewWhatisthefunctionofbonemarrow骨髓Hematopoiesis造血Redbloodcells---transportoxygenandnutrients

Whitebloodcells---fightinfection

platelets—helpbloodclot凝结Bonemarrowhaematopoiesisfailure,HSC造血干细胞injuredPeripheralbloodpancytopenia全血细胞减少Typicalsymptoms:anemia,infection,bleedingDirectandindirectpathophysiologicpathwayscausingA.AThemaincharactersofAplasticAnemiaGeneralIntroductionClassification

ⅠⅡSevereModerateAbroadAcuteChronicHomeEpidemiologyIncidence

China

Annualincidence

7.4per100,000

6.01.4DistributionEpidemiologyfemalemaleInfant70’sChemicalfactors:drug

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

chemicals

(benzene苯,insecticides杀虫剂etc)Physicalfactors

radiation,XrayBiologicalfactors

viruses,severebacterium,infectionOtherpathogenicfactorsUnknownEtiologyPathogenesisFailureinhematopoieticstemorprogenitor祖cellsDefectinmicroenvironmentofhaematopoiesisImmuno-mediated免疫介导的

bonemarrowfailureGeneticaspects:HLAassociatedsusceptibility敏感SeedsSoilHarmfulInsectsPresentingsymptomsAcuteAAChronicAAOnsetacute,shorthistoryslowlyonset,longerhistoryAnemiasevere,remarkable显著的relativelymoderateBleedingheavybleeding,Multiorgan多器官Hemorrhage出血moderate,skinandmucusInfectionmorefrequent,moderate,upperrespiratoryinfectionSepsis败血症Laboratoryfindings

Bloodcount（x109/L）

granulocytes<0.5>0.5

platelets<20>20

reticulocytes<15>15

BMmorphology骨髓形态学AcuteAAChronicAAmultiplacehypocellularitylackofmegakaryocyteslackofE,Gincreaseinmarrowfatincreasednon-HCSimilarfindings,focalareaactivehaematopoiesisLaboratoryfindings

Bloodcount（x109/L）

粒细胞<0.5>0.5

血小板<20>20

网织红细胞<15>15

BMmorphology骨髓形态学AcuteAAChronicAA多种细胞减少巨核细胞缺乏红细胞、粒细胞缺乏

骨髓脂肪增多非造血细胞增多相似表现,核心区域造血活跃Bonemarrowaspiration吸NormalBMbiopsy

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

Notethepresenceofmegakaryocytes,erythroidislands,andgranulocyticprecursors粒细胞前体细胞.Thismarrowistakenfromtheposterioriliaccrest髂后上嵴inamiddleagedperson,soitisabout50%cellular,withsteatocytesadmixedwiththemarrowelements.AplasticanemiabiopsyHematopoieticelementsinthisbonemarrowbiopsyaremarkedly明显的reduced.Ofcourse,RBC,plateletsandgranulocyteswilloftenbediminished减少的.MarrowFatAplasticanemia(HP)BMmorphologyofAcuteAARecoverphase恢复阶段DiagnosticstandardofAA(1987)

DiagnosisDiagnosisSignBMPeriBlood外周血Exclusion排除DiagnosticstandardofAA(1987)

Decreasedperipheralbloodcount，absolutenumber绝对数量ofreticulocytesreduced

Withoutmegalosplenia脾肿大

DiagnosisBMmorphology

DiagnosisModerateorseverehypocellularity细胞缺乏foundatleastoneaspiration(biopsy)site.Nonhaematopoieticcells非造血细胞increaseinBM.Otherdiseaseswhichmaycausedecreasedbloodcountarecarefullyexcluded，suchasmyelodysplasticsyndrome骨髓增生异常综合症(MDS),acutemyelogenousleukemia急性骨髓性白血病(AML).

Routinetherapiesforanemiafailtowork.DiagnosisMyelodysplasticsyndrome--dysmorphic异形的featuresParoxysmalnocturnalhaemoglobinuria阵发性睡眠性血红蛋白尿(PNH)HypoplasticAcuteLeukemia低增生性急性白血病OthersDifferentialDiagnosis–Pancytopenia全血细胞减少Anacquiredhaematopoieticstemcelldefectwithpredominant主要的haemolyticanaemia溶血性贫血.Adescriptiveterm描述词fortheclinicalmanifestationofhaemolysis溶血andhaemoglobinuria血红蛋白尿manifestbydarkcolouredurineinthemorning.PNH--ParoxysmalnocturnalhaemoglobinuriaParoxysmalnocturnalhaemoglobinuria.Historically历史上的testwasHam’stest;showedredcelllysis溶解bycomplement补体activationinacidified酸化的serum.Currentlytestforabsentproteinsoncellsurface.CD55andCD59TreatmentSupportiveCareGrowthHormonesImmuneSuppressiveTherapyHematopoieticStemCellTransplantationRedcelltransfusionPlatelettransfusionManagementofneutropenia中性粒细胞减少privateroomfacemaskhandwashbroad-spectrumantibiotics广谱抗生素iffeverSupportiveCareIndicatedforpatients>50yearsPatientswithnoHLAmatchedsiblingdonors同胞捐助者Anti-ThymocyteGlobulin抗胸腺细胞球蛋白(ATG)oranti-lymphocyteglobulin抗人淋巴细胞球蛋白(ALG),cyclosporin,methylprednisoneBestresultsareforcombinationtherapy.Responseisslow,4-12weekstoseeearlyimprovementImmunosuppressivetherapyResponserates60-70%Relapses复发arecommonandcontinuedsupportivecareneeded.Upto50%ofrelapsedpatientswillrespondto2ndcourseofimmunosuppressivetherapyAge<55years.Conditioning调节withCyclophosphamide环磷酰胺&ant