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- 2022-05-26 16:47:28 发布
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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
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