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1、NK/T細(xì)胞淋巴瘤包括：結(jié)外NK/T細(xì)胞淋巴瘤，鼻型侵襲性NK細(xì)胞白血病慢性NK細(xì)胞淋巴增生性疾病NK細(xì)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-likeTcellsDiscrepan
2、cyofCD3expression inNKcellsCD3isaTCR-associatedcomplexFormedby6chains:?,?,2?,2?NKcellshavecytoplasmic?or?,butcannotassembleintactCD3complexConventionalCD3antibodydetects?chain“cytoplasmicCD3”SurfaceCD3(asdetectedbyLeu4/OKT3)byflowcytometryoronfrozentissueisNEGATIVETCRLeu4,OKT3結(jié)外NK/T細(xì)胞
3、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(jié)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，常需多次取材才能確診。大多數(shù)病例為EBV（+）、CD56（+）的NK細(xì)胞表型，而少數(shù)病例具有EBV（+）、CD56（-）的細(xì)胞毒性T細(xì)胞表型。SmallMedium-sizedLargeBroadcytologicspectrumMorphologicfeaturesSurfacesquamousepith
4、eliumcanshowfloridpseudoepitheliomatoushyperplasiaEntrappednasalmucosalglandsoftenshowclearcellchangeSkin:infiltrateoftenperivascularandperiadnexal,butsometimesdiffusedermal;subcutiscanbeinvolvedIntestine:UlcerationandperforationcommonSkinIntestineTestisMuscle結(jié)外NK/T細(xì)胞淋巴瘤的免疫表型CD2+表面CD3
5、-;胞漿CD3+(polyclonalCD3?;PS1)CD56+,CD57和CD16常-CD4,CD5,CD7,CD8:常-CD43,CD45RO:常+細(xì)胞毒顆粒+Ki67:高CD25和CD30偶爾+TCR-;CD3CD5CD56GranzymeBExtranodalNK/Tcelllymphoma:GenotypeTCRgenes:germlineinmostcasesstudiedEBVPresentinclonalepisomalformNasal:>95%positive,irrespectiveofethnicoriginExtra-nasal:>90
6、%positiveinAsians,butlower%inCaucasiansExtranodalNK/Tcelllymphoma:MainproblemsindiagnosisBiopsiesmayshowextensivecoagulativenecrosis,renderingitimpossibletomakeadefinitivediagnosis.Repeatbiopsyisrequired.Someexamplescomprisesmallcellswithminimalcytologicatypia:distinctionfromreactivel
7、ymphoidinfiltrateverydifficultBEWARE:Mucosalsmalllymphoidcellsoftenappearslightlylargerthansmalllymphocytes,andcanshowirregularnucleiHistologicfeaturesfavoringaneoplasticprocessAbnormalarchitecture:DenseexpansileinfiltratewithwideseparationandlossofmucosalglandsTissueulcerationandnecr
8、osisE
