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1、HowwediagnoseSAADiagnosisanddifferentialdiagnosisPatientswithSAAusual1yhavebeenpreviouslywel1,priortothediagnosis.Fortheconsultanthematologist，thedifferentialwillmainlylieamonghematologicsyndromes,usuallydistinguishableonbonemarrowexamination.Thereaderi
2、sreferredtogeneralreferencesforacompletelistofdiseasesthatcanpresentwithvariousdegreesofcytopenias.-Anelevatedmeancorpuscularvolumeisfrequentinaplasticanemiaatpresentation.The“empty”marrowonhistologyofSAAishighlycharacteristicandarequisiteforthediagnosi
3、s.Markedhemophagocytosis,obviousdysplasia,orincreasedblastsindicateotherdiseases,althoughdifferentiationofhypocellularmyelodysplasticsyndrome(seenin〜20%ofmyelodysplasticsyndrome[MDS]cases)fromaplasticanemiacanbedifficuIt.Megakaryocytesarethemostreliable
4、lineagetouseindistinguishingMDSfromSAA:smallmononuclearoraberrantmegakaryocytesaretypicalofMDS,whereasmegakaryocytesaremarkedlyreducedorabsentinSAA.Incontrast,umega1ob1astoidvandmodestdysplasticerythropoiesisarenotuncommoninanaplasticmarrow,especiallywh
5、enaparoxysmalnoctuimalhemoglobinuria(PNH)cloneispresent.CytogeneticsarehelpfulwhentypicalofMDS,butsomeaberrations(suchastrisomy6,trisomy8,and13q-)mayappearinSAAthatisresponsiveto1ST,accordingtosomereports,--butnotconfirmedbyothers-(forourSAAresearchprot
6、ocols,abnormalchromosomesareanexclusioncriterion)•AplasticanemiaandPNHoverlapinapproximately40%to50%ofcases(theAA/PNHsyndrome).-Atourinstitution,morethan1%granulocytesdeficientinglycos)dphosphoinsoitol-linkedproteinsdetectablebyf1owcytometryareconsidere
7、dabnormal,butothermethodologiescandetectevensmal1erPNIIclones.Suchsmallclonesdonotresultinsignificanthemolysisorriskofthrombosis,andwhetherthepresenceofasmallPNHcloneinthesettingofhypocellularmarrowfailurehasclinicalsignificanceorpredictsresponsetotreat
8、mentandoutcomesiscontroversial--Thepreseneeoflessthan50%glycosylphosphatidvlinositol-deficientcirculatingcellswithoutevidenceforthrombosisorsignificanthemolysisgenerallydoesnotrequirePNH-specifictherapy.-IrrespectiveofPNHclonesiz
