再生障礙性貧血英文專家講座

Definition

Aplasticanemiaisalife-threateningsyndromecharacterizedbyfailureofthebonemarrowtoproduceperipheralbloodcellsandtheirprogenitors.再生障礙性貧血英文專家講座第1頁EtiologyandpathogenesisThemajorcausesforaplasticanemia:1.Infections(e.g.Hepatitis,Epstein-BarrvirusandParvovirusB19).2.RadiationXrays,γrays3.Chemicals:benzene4.Drugs:Chloramphenicol(氯霉素)再生障礙性貧血英文專家講座第2頁Immune-Mediatedinjuryisthemainpathogenesis.Inaddition,absentordefectivehematopoieticstemcellsandabnormalbonemarrowmicroenviromentcanalsocauseaplasticanemia.再生障礙性貧血英文專家講座第3頁ClinicalmanifestationandclassificationClinicalmanifestationAnemia,BleedingandInfectionsarecommonsymptomsClassification1.SevereAplasticanemia（SAA）2.Non-severeAplasticanemia（NSAA）再生障礙性貧血英文專家講座第4頁PhysicalExamination1.Petechiaeandecchymosesaretypical.2.Palloroftheskinandmucousmembranesiscommon.再生障礙性貧血英文專家講座第5頁LaboratoryStudies1.BloodSmear:Pancytopenia(anemia,leukopenia,andthrombocytopenia)isauniversalpresentingfinding.SAAbloodsmearNormalperipheralbloodsmear再生障礙性貧血英文專家講座第6頁2.bonemarrowbiopsy:Biopsyissuperiorfordeterminationofcellularityandshowsmainlyfatunderthemicroscope,withhematopoieticcellsoccupying<25%ofthemarrowspace;inthemostseriouscasesthebiopsyisvirtually100%fat.Itisrequiredtoestablishthediagnosis.再生障礙性貧血英文專家講座第7頁A.Normalbonemarrowbiopsy.Thisspecimenshowsanabundanceofhematopoieticcells,includingMyeloidanderythroidprecursorsandNormal-appearingmegakaryocytesB.Aplasticanemiabiopsy.Thisspecimenshowsfewdetectablehematopoieticcells,andthosethatcanbeseen(onesmallnest)arelargelylymphocytes.再生障礙性貧血英文專家講座第8頁3.BonemarrowsmearThebonemarrowisusuallyreadilyaspiratedbutdiluteonsmear,andthefattybiopsyspecimenmaybegrosslypaleonwithdrawal;Insevereaplasiathesmearoftheaspiratedspecimenshowsonlyredcells,residuallymphocytes,andstromalcells.再生障礙性貧血英文專家講座第9頁DD.Marrowsmearinaplasticanemia.Themarrowshowsreplacementofhematopoietictissuebyfatandonlyresidualstromalandlymphoidcells.C.Normalbonemarrowaspiratesmear.Themarrowisnormally30–70%cellular,andthereisaheterogeneousmixofmyeloid,erythroid,andlymphoidcells.C再生障礙性貧血英文專家講座第10頁ReferencestandardofdiagnosisforAA1.Peripheralbloodpancytopenia,reticulocytepercentagelessthan0.01andtheratioofLymphocyteisincreased.

2.Generally,withouttheliverandspleenenlargement.3.Atleastoneareaofhypoplasiainbonemarrow.Non-hematopoieticcellsincrease.4.EliminationofotherdiseaseswhichmaycausepancytipeniasuchasPNH,MDS,acutearrestofhemopoiesis,etc.5.Commontherapyforanemiaisineffective.再生障礙性貧血英文專家講座第11頁SAAlaboratorycriteriaTodefineSAAtheremustbeatleasttwoofthefollowing:(1)absoluteneutrophilcountlessthan0.5×109/L,(2)plateletcountlessthan20×109/L,or(3)reticulocyteslessthan20×109/L.Non-severeAA:Patientsnotfulfillingthecriteriaforsevereaplasticanaemia.再生障礙性貧血英文專家講座第12頁TreatmentHematopoieticStemCellTransplantation

Thisisthebesttherapyfortheyoungpatientwithafullyhistocompatiblesiblingdonor.Immunosuppr