nk细胞淋巴瘤.ppt

NK/T细胞淋巴瘤,包括：结外NK/T细胞淋巴瘤，鼻型侵袭性NK细胞白血病慢性NK细胞淋巴增生性疾病,NK细胞的免疫表型特点,NosinglemarkerunequivocallyidentifiesallNKcells,CD270-90%s-CD30%c-CD395%CD40%CD50%CD780-90%CD830-40%,CD11b*80-90%CD16*80-90%CD56*95%CD57*50-60%CD2595%,*CommonlyusedNKmarkers,butexpressedinbothNKcellsandNK-likeTcells,DiscrepancyofCD3expressioninNKcells,CD3isaTCR-associatedcomplexFormedby6chains:,2,2NKcellshavecytoplasmicor,butcannotassembleintactCD3complexConventionalCD3antibodydetectschain“cytoplasmicCD3”,SurfaceCD3(asdetectedbyLeu4/OKT3)byflowcytometryoronfrozentissueisNEGATIVE,TCR,Leu4,OKT3,结外NK/T细胞淋巴瘤，鼻型,a鼻腔、鼻咽部、鼻窦及腭部最常见，皮肤、软组织、胃肠道和睾丸亦常见。b鼻腔者表现为鼻塞、鼻衄，粘膜坏死及溃疡。皮肤病变表现为结节并多伴有溃疡。发生在肠道者常发生穿孔。c肿瘤常有噬血管性，多伴有血管破坏和坏死。d常坏死明显，活检取材要取及非坏死瘤组织，常需多次取材才能确诊。,大多数病例为EBV（+）、CD56（+）的NK细胞表型，而少数病例具有EBV（+）、CD56（-）的细胞毒性T细胞表型。,Small,Medium-sized,Large,Broadcytologicspectrum,Morphologicfeatures,SurfacesquamousepitheliumcanshowfloridpseudoepitheliomatoushyperplasiaEntrappednasalmucosalglandsoftenshowclearcellchangeSkin:infiltrateoftenperivascularandperiadnexal,butsometimesdiffusedermal;subcutiscanbeinvolvedIntestine:Ulcerationandperforationcommon,Skin,Intestine,Testis,Muscle,结外NK/T细胞淋巴瘤的免疫表型CD2+表面CD3-;胞浆CD3+(polyclonalCD3;PS1)CD56+,CD57和CD16常-CD4,CD5,CD7,CD8:常-,CD43,CD45RO:常+细胞毒颗粒+Ki67:高CD25和CD30偶尔+TCR-;,CD3,CD5,CD56,GranzymeB,ExtranodalNK/Tcelllymphoma:Genotype,TCRgenes:germlineinmostcasesstudied,EBVPresentinclonalepisomalformNasal:95%positive,irrespectiveofethnicoriginExtra-nasal:90%positiveinAsians,butlower%inCaucasians,ExtranodalNK/Tcelllymphoma:Mainproblemsindiagnosis,Biopsiesmayshowextensivecoagulativenecrosis,renderingitimpossibletomakeadefinitivediagnosis.Repeatbiopsyisrequired.Someexamplescomprisesmallcellswithminimalcytologicatypia:distinctionfromreactivelymphoidinfiltrateverydifficult,BEWARE:Mucosalsmalllymphoidcellsoftenappearslightlylargerthansmalllymphocytes,andcanshowirregularnuclei,Histologicfeaturesfavoringaneoplasticprocess,Abnormalarchitecture:DenseexpansileinfiltratewithwideseparationandlossofmucosalglandsTissueulcerationandnecrosisEvidenceofinvasion:AngiocentricgrowthCytologicatypia:ClearcytoplasmMedium-sized;nuclearirregularities;granularchromatinReadilyfoundmitoticfiguresinasmallcelllesion,Immunohistochemicalanalysis:First-tierpanel,CD20,CD3,CD56,(Ki67),NodularaggregatesofBcellsseparatedbyTcells;fewCD56+cells,Reactivelymphoidinfiltrate,DensesheetsofBcells,Bcelllymphoma,DensesheetsofCD3+CD56+cells,NK/Tcelllymphoma,DensesheetsofCD3+CD56-cells,ReactiveorCD56-NK/TlymphomaorTcelllymphoma,HowtoconfirmasuspicionofNK/Tcelllymphoma?,ImmunostainforCD56:largeclustersorsheetsofpositivecellsstronglysupportdiagnosisoflymphomaPitfall:herpesinfectionIn-situhybridizationforEBER:largegroupsofpositivecellssupportdiagnosisoflymphomaImmunostainforKi67:High