nk细胞淋巴瘤讲义课件

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

DiscrepancyofCD3expression

inNKcellsCD3isaTCR-associatedcomplexFormedby6chains:,,2,2NKcellshavecytoplasmicor,butcannotassembleintactCD3complexConventionalCD3antibodydetectschain“cytoplasmicCD3”SurfaceCD3(asdetectedbyLeu4/OKT3)byflowcytometryoronfrozentissueisNEGATIVETCRLeu4,OKT3结外NK/T细胞淋巴瘤，鼻型

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。MorphologicfeaturesSurfacesquamousepitheliumcanshowfloridpseudoepitheliomatoushyperplasiaEntrappednasalmucosalglandsoftenshowclearcellchangeSkin:infiltrateoftenperivascularandperiadnexal,butsometimesdiffusedermal;subcutiscanbeinvolvedIntestine:UlcerationandperforationcommonSkinIntestineTestisMuscle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-;CD3CD5CD56GranzymeBExtranodalNK/Tcelllymphoma:GenotypeTCRgenes:germlineinmostcasesstudiedEBVPresentinclonalepisomalformNasal:>95%positive,irrespectiveofethnicoriginExtra-nasal:>90%positiveinAsians,butlower%inCaucasiansExtranodalNK/Tcelllymphoma:MainproblemsindiagnosisBiopsiesmayshowextensivecoagulativenecrosis,renderingitimpossibletomakeadefinitivediagnosis.Repeatbiopsyisrequired.Someexamplescomprisesmallcellswithminimalcytologicatypia:distinctionfromreactivelymphoidinfiltrateverydifficultBEWARE:Mucosalsmalllymphoidcellsoftenappearslightlylargerthansmalllymphocytes,andcanshowirregularnucleiHistologicfeaturesfavoringaneoplasticprocessAbnormalarchitecture:DenseexpansileinfiltratewithwideseparationandlossofmucosalglandsTissueulcerationandnecrosisEvidenceofinvasion:AngiocentricgrowthCytologicatypia:ClearcytoplasmMedium-sized;nuclearirregularities;granularchromatinReadilyfoundmitoticfiguresinasmallcelllesionImmunohistochemicalanalysis:

First-tierpanelCD20,CD3,CD56,(Ki67)NodularaggregatesofBcellsseparatedbyTcells;fewCD56+cellsReactivelymphoidinfiltrateDensesheetsofBcellsBcelllymphomaDensesheetsofCD3+CD56+cellsNK/TcelllymphomaDensesheetsofCD3+CD56-cellsReactiveorCD56-NK/TlymphomaorTcelllymphomaHowtoconfirmasuspicionof

NK/Tcelllymphoma?ImmunostainforCD56:largeclustersorsheetsofpositivecellsstronglysupportdiagnosisoflymphoma[Pitfall:herpesinfection]In-situhybridizationforEBER:largegroupsofpositivecellssupportdiagnosisoflymphomaImmunostainforKi67:Highindex(>80%)fora