再生障碍性贫血(英文PPT).ppt 16页

Aplasticanemia(AA) DefinitionAplasticanemiaisalife-threateningsyndromecharacterizedbyfailureofthebonemarrowtoproduceperipheralbloodcellsandtheirprogenitors. EtiologyandpathogenesisThemajorcausesforaplasticanemia:1.Infections(e.g.Hepatitis,Epstein-BarrvirusandParvovirusB19).2.RadiationXrays,γrays3.Chemicals:benzene4.Drugs:Chloramphenicol(氯霉素) Immune-Mediatedinjuryisthemainpathogenesis.Inaddition,absentordefectivehematopoieticstemcellsandabnormalbonemarrowmicroenviromentcanalsocauseaplasticanemia. ClinicalmanifestationandclassificationClinicalmanifestationAnemia,BleedingandInfectionsarecommonsymptomsClassification1.SevereAplasticanemia（SAA）2.Non-severeAplasticanemia（NSAA） PhysicalExamination1.Petechiaeandecchymosesaretypical.2.Palloroftheskinandmucousmembranesiscommon. LaboratoryStudies1.BloodSmear:Pancytopenia(anemia,leukopenia,andthrombocytopenia)isauniversalpresentingfinding.SAAbloodsmearNormalperipheralbloodsmear 2.bonemarrowbiopsy:Biopsyissuperiorfordeterminationofcellularityandshowsmainlyfatunderthemicroscope,withhematopoieticcellsoccupying<25%ofthemarrowspace;inthemostseriouscasesthebiopsyisvirtually100%fat.Itisrequiredtoestablishthediagnosis. A.Normalbonemarrowbiopsy.Thisspecimenshowsanabundanceofhematopoieticcells,includingMyeloidanderythroidprecursorsandNormal-appearingmegakaryocytesB.Aplasticanemiabiopsy.Thisspecimenshowsfewdetectablehematopoieticcells,andthosethatcanbeseen(onesmallnest)arelargelylymphocytes. 3.BonemarrowsmearThebonemarrowisusuallyreadilyaspiratedbutdiluteonsmear,andthefattybiopsyspecimenmaybegrosslypaleonwithdrawal;Insevereaplasiathesmearoftheaspiratedspecimenshowsonlyredcells,residuallymphocytes,andstromalcells. DD.Marrowsmearinaplasticanemia.Themarrowshowsreplacementofhematopoietictissuebyfatandonlyresidualstromalandlymphoidcells.C.Normalbonemarrowaspiratesmear.Themarrowisnormally30–70%cellular,andthereisaheterogeneousmixofmyeloid,erythroid,andlymphoidcells.C ReferencestandardofdiagnosisforAA1.Peripheralbloodpancytopenia,reticulocytepercentagelessthan0.01andtheratioofLymphocyteisincreased.2.Generally,withouttheliverandspleenenlargement.3.Atleastoneareaofhypoplasiainbonemarrow.Non-hematopoieticcellsincrease.4.EliminationofotherdiseaseswhichmaycausepancytipeniasuchasPNH,MDS,acutearrestofhemopoiesis,etc.5.Commontherapyforanemiaisineffective. SAAlaboratorycriteriaTodefineSAAtheremustbeatleasttwoofthefollowing:(1)absoluteneutrophilcountlessthan0.5×109/L,(2)plateletcountlessthan20×109/L,or(3)reticulocyteslessthan20×109/L.Non-severeAA:Patientsnotfulfillingthecriteriaforsevereaplasticanaemia. TreatmentHematopoieticStemCellTransplantationThisisthebesttherapyfortheyoungpatientwithafullyhistocompatiblesiblingdonor.ImmunosuppressionSupportiveCareOtherTherapies Apathwayforthediagnosticmanagementofpatientswithaplasticanemia Thankyou!