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1、PathogenesisandTreatmentofExtranodalNaturalKiller/T-CellLymphomaSeminHematol.2014,51:42–51.——LiYePATHOGENESISOFNK/T-CELLLYMPHOMA1.Chromosomalabnormalities2.Geneticaberrations3.TheEpstein-Barrvirus(EBV)ChromosomalabnormalitiesDeletionofthelongarmofchromosome6BrJHaematol.1997;98:922-6.Chromo
2、somalabnormalitiesBrJHaematol.1997;97:621-5.GeneticaberrationsFOXO3,PRDM1,TP53,CDKN2A,FAS,SHP1,TP73,andKITJClinExpHematopathol.2005;45:51-70.PATHOLOGYOFNK/T-CELLNEOPLASMS1.diffuseproliferationoflymphomacellswithanangiocentricorangiodestructivegrowthpattern2.Infiltrationofinflammatorycellsa
3、ndsometimesaccompanynecroticchanges3.NKcellmarkers(CD2,cytoplasmicCD3(cyCD3),CD7,andCD56)4.CytotoxicmoleculessuchasTIA-1,granzymeB,andperforinDISEASEFEATURESOFNK/T-CELLLYMPHOMA1.originforENKL:thenoseandparanasalareaskin,gastrointestinaltract,etal.2.StageI/II:Approximatelyhalfofthepatients3
4、.NasalandextranasalENKLsinthesamecategoryofdiseaseaccordingtothecurrentWHOclassificationAnnOncol.2010;21:1032-40.NasalENKLmorefrequentlypresentsasalocalizeddisease,whereasextranasalENKLismorefrequentlydetectedatanadvancedstage.Blood.2009;113:3931-3937A.Limited-stage(I/II)diseaseB.advanced-
5、stage(III/IV)diseaseBlood.2009;113:3931-3937TREATMENTOFEXTRANODALNK/T-CELLLYMPHOMA1.LimitedStages2.AdvancedStages,RelapsedorRefractoryState3.HematopoieticStemCellTransplantation(autologousandallogeneicHSCTs)Cancer.1995;76:2351-6.theexpressionofP-glycoprotein(P-gp)/MDRltheproductofthemultid
6、rugresistance(MDR)1geneβ-actinMDR3MDRlDrug:vincristineanddoxorubicinJClinOncol.2000;18:54-63.JClinOncol.2000;18:54-63.(median56months)Treatmentfailure57/92(62%)(median8months)Relapsedrate14/61(23%)AnnOncol.2001;12:349-52.Newtreatmentstrategies,suchashigh-dosechemotherapywithstem-celltransp
7、lantation,earlyadministrationofradiationtreatment,orconcomitantchemo-radiotherapy,needtobeinvestigatedtoimprovethetreatmentoutcomes,particularlyinpatientswithpatientswithBsymptoms.1.Theplannedsequentialchemo-radiotherapy6/17(35%)2.Estimatedoverallthree-yearsur
