nk細胞淋巴瘤講義 課件

NK/T細胞淋巴瘤包括：結外NK/T細胞淋巴瘤，鼻型侵襲性NK細胞白血病慢性NK細胞淋巴增生性疾病NK細胞的免疫表型特點

NosinglemarkerunequivocallyidentifiesallNKcellsCD270-90%s-CD30%c-CD3

>95%CD40%CD50%CD780-90%CD830-40%CD11b*80-90%CD16*80-90%CD56*>95%CD57*50-60%CD25>95%*CommonlyusedNKmarkers,butexpressedinbothNKcellsandNK-likeTcellsDiscrepancyofCD3expression

inNKcellsCD3isaTCR-associatedcomplexFormedby6chains:,,2,2NKcellshavecytoplasmicor,butcannotassembleintactCD3complexConventionalCD3antibodydetectschain“cytoplasmicCD3〞SurfaceCD3(asdetectedbyLeu4/OKT3)byflowcytometryoronfrozentissueisNEGATIVETCRLeu4,OKT3結外NK/T細胞淋巴瘤，鼻型

a鼻腔、鼻咽部、鼻竇及腭部最常見，皮膚、軟組織、胃腸道和睪丸亦常見。b鼻腔者表現(xiàn)為鼻塞、鼻衄，粘膜壞死及潰瘍。皮膚病變表現(xiàn)為結節(jié)并多伴有潰瘍。發(fā)生在腸道者常發(fā)生穿孔。c腫瘤常有噬血管性，多伴有血管破壞和壞死。d常壞死明顯，活檢取材要取及非壞死瘤組織，常需屢次取材才能確診。大多數病例為EBV〔+〕、CD56〔+〕的NK細胞表型，而少數病例具有EBV〔+〕、CD56〔-〕的細胞毒性T細胞表型。SmallMedium-sizedLargeBroadcytologicspectrumMorphologicfeaturesSurfacesquamousepitheliumcanshowfloridpseudoepitheliomatoushyperplasiaEntrappednasalmucosalglandsoftenshowclearcellchangeSkin:infiltrateoftenperivascularandperiadnexal,butsometimesdiffusedermal;subcutiscanbeinvolvedIntestine:UlcerationandperforationcommonSkinIntestineTestisMuscle結外NK/T細胞淋巴瘤的免疫表型CD2+外表CD3-;胞漿CD3+(polyclonalCD3;PS1)CD56+,CD57和CD16常-CD4,CD5,CD7,CD8:常-CD43,CD45RO:常+細胞毒顆粒+Ki67:高CD25和CD30偶爾+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: