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1、AplasticAnemia(AA)ZhongshanHospital,FudanUniversityZouShanhua。OutlineBonemarrowhematopoiesisfailureledbychemicals,radiation,biologicalfactorsandunknownetiology.Thecommonclinicalpresentationismildtoseverepancytopenia.EpidemiologyTheincidencerateofAA:0.74per100,000,AAA:0.14per100,0
2、00,CAA:0.60per100,000.Distribution:mostAApatientsareyoungandmiddle-agedpeople,malemorethanfemaleEtiologyPrimaryAA:unknowncauses,about60~70%SecondaryAAEtiologyofSecondaryAAChemicalsDrug:antibiotics,antineoplasticagent,NSAIDsOtherchemicals:benzeneandbenzenederivatives,arsenic,heavy
3、metals,insecticidesEtiologyofSecondaryAARadiationViralinfection:Hepatitisvirus,EBVMechanismHematopoieticstemcelldefectStemcellscountdecreaseStemcellsproliferationcapacitydecreaseMechanismHematopoieticmicroenviromentdefectstromalcellshematopoieticgrowthfactormicrocirculationMechan
4、ismImmunologicderangementCD4/CD8(↓,inversion)γ-IFN,TNFα,IL-2↑immunosuppressivetherapyClinicalFeaturesClinicalfeatures:anemia,infection,hemorrhageDifferences:AAA(SAA-Ⅰ),CAA,SAA-ⅡLaboratorystudy—completebloodcountCommonfeature:anemia,reticulocytecount↓granulocytopeniathrombocytopen
5、iapercentoflymphocyteincreaserelativelyLaboratorystudy—completebloodcountSAA:Ret<1%Granulocytecount<0.5×109/LBPC<20×109/LCAA:Ret>1%Granulocytecount>0.5×109/LBPC>20×109/LLaboratorystudy-bonemarrowCommonfeature:Nakedeye:bonemarrowisplentyofoildropsMicroscope:hypoplasiaorpolarhypopl
6、asia;hematopoieticcellsdecrease,nonhematopoieticcellsincreaseLaboratorystudy-bonemarrowSAA:polarhypoplasia,progenitorcellcanhardlybeseen,nomegakaryocyteCAA:hypoplasia,megakaryocytesdecreaseordisappearLaboratorystudy-bonemarrowbiopsyMorevaluablethanmarrowaspirationRatioofhematopoi
7、etictissue/fattytissueMegakaryocyteproliferationcanbeevaluatedaccurately;LaboratorystudyCFU-GM↓NAP↑EPO↑CriteriaofdiagnosisPancytopenia,reticulocyteabsolutecount↓WithoutsplenomegalyHypoplasiaorpolarhypoplasiaofbonemarrow;ifnot,megakaryocyteshoulddisappear;Biopsy:hematopoietictissu
8、e↓fattytissue↑Excludingotherdiseaseswith