nk细胞淋巴瘤讲义课件

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1、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、cyofCD3expressioninNKcellsCD3isaTCR-associatedcomplexFormedby6chains:,,2,2NKcellshavecytoplasmicor,butcannotassembleintactCD3complexConventionalCD3antibodydetectschain“cytoplasmicCD3”SurfaceCD3(asdetectedbyLeu4/OKT3)byflowcytometryoronfrozentissueisNEGATIVETCRLeu4,OKT3结外NK/T细胞

3、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。SmallMedium-sizedLargeBroadcytologicspectrumMorphologicfeaturesSurfacesquamousepith

4、eliumcanshowfloridpseudoepitheliomatoushyperplasiaEntrappednasalmucosalglandsoftenshowclearcellchangeSkin:infiltrateoftenperivascularandperiadnexal,butsometimesdiffusedermal;subcutiscanbeinvolvedIntestine:UlcerationandperforationcommonSkinIntestineTestisMuscle结外NK/T细胞淋巴瘤的免疫表型CD2+表面CD3

5、-;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

6、%positiveinAsians,butlower%inCaucasiansExtranodalNK/Tcelllymphoma:MainproblemsindiagnosisBiopsiesmayshowextensivecoagulativenecrosis,renderingitimpossibletomakeadefinitivediagnosis.Repeatbiopsyisrequired.Someexamplescomprisesmallcellswithminimalcytologicatypia:distinctionfromreactivel

7、ymphoidinfiltrateverydifficultBEWARE:Mucosalsmalllymphoidcellsoftenappearslightlylargerthansmalllymphocytes,andcanshowirregularnucleiHistologicfeaturesfavoringaneoplasticprocessAbnormalarchitecture:DenseexpansileinfiltratewithwideseparationandlossofmucosalglandsTissueulcerationandnecr

8、osisE