NK细胞增殖性疾病同济医院血液内科周剑峰2015年06月07日T和NK细胞肿瘤的分类：WHO2008WHO2008:thematureT-cellandNK-cellneoplasmsT-cellprolymphocyticleukemiaT-celllargegranularlymphocyticleukemiaChroniclymphoproliferativedisorderofNK-cells*AggressiveNKcellleukemiaSystemicEBV+T-celllymphoproliferativediseaseofchildhood(associatedwithCAEBV)Hydroavacciniforme-likelymphomaAdultT-cellleukemia/lymphomaExtranodalNK/Tcelllymphoma,nasaltypeEnteropathy-associatedT-celllymphomaHepatosplenicT-celllymphomaSubcutaneouspanniculitis-likeT-celllymphomaMycosisfungoidesSézarysyndromePrimarycutaneousCD30+T-celllymphoproliferativedisorderLymphomatoidpapulosisPrimarycutaneousanaplasticlarge-celllymphomaPrimarycutaneousaggressiveepidermotropicCD8+cytotoxicT-celllymphoma*Primarycutaneousgamma-deltaT-celllymphomaPrimarycutaneoussmall/mediumCD4+T-celllymphoma*PeripheralT-celllymphoma,nototherwisespecifiedAngioimmunoblasticT-celllymphomaAnaplasticlargecelllymphoma(ALCL),ALK+Anaplasticlargecelllymphoma(ALCL),ALK−*2001WHO2008WHOCommentsAngioimmunoblasticLymphomaAngioimmunoblasticLymphomaDefinitionoforigincellAnaplasticLargeCellLymphoma2variantsbasedonALK(+/-)expressionPrognosticimportanceUnspecifiedPeripheralT-cellLymphomaPeripheralT-cellLymphomasnotOtherwiseSpecified3variants:lymphoepitelioidlymphoma,Tzonelymphoma(2001WHO)andfollicularlymphoma(2008WHO)T/NK-celllymphoma,nasaltypeT/NK-celllymphoma,nasaltypeNochangesEntheropathy-associatedT-celllymphomaEntheropathy-associatedT-celllymphomasTwovariants:classicalandmonomorphictypeswithgeneticchangescommontobothHepatosplenicT-celllymphomaHepatosplenicT-celllymphomaNochangesSubcutaneouspanniculitis-likeT-celllymphomaSubcutaneouspanniculitis-likeT-celllymphomaOnlyabandassociatedwithautoimmunedisorderMycosisfungoidesMycosisfungoidesNewstagingandnewinformationaboutpathogenesisSézarysyndromeSézarysyndromeNewmarkersPrimarycutaneousanaplasticlargecelllymphomaPrimarycutaneousanaplasticlargecelllymphomaRecognitionofCD8+casesLymphomatoidpapulosisLymphomatoidpapulosisThreehistologicaltypesPrimarycutaneousgamma-deltaT-celllymphomaThreehistopathologicpatterns:epidermotropic,dermic,andsubcutaneoussubtypesPrimarycutaneousCD8+aggressiveepidermotropiccytotoxicT-celllymphomaProvisionalentityPrimarycutaneousCD4+small/mediumT-celllymphomaProvisionalentityBlasticNK-celllymphomaPlasmocytoiddendriticcellneoplasmNowitisoneofthemyeloidneoplasmsT-cellprolymphocyticleukemiaT-cellprolymphocyticleukemiaNochangesT-celllargegranularlymphocyticleukemiaT-celllargegranularlymphocyticleukemiaNewetiologicalfeaturesandnewmarkersChroniclymphoproliferativedisorderofNK-cellsProvisionalentityAggressiveNK-cellleukemiaAggressiveNK-cellleukemiaNochangesAdultT-cellleukemia/lymphomaAdultT-cellleukemia/lymphomaDefinitionoftheregulatoryT-cellnormalcounterpartT和NK细胞肿瘤分类的主要变化EBV相关淋巴增殖性疾病JKoreanMedSci.2008Apr;23(2):185-92.EBV相关T/NK细胞增殖性疾病JDermatol.2014;41(1):29-39.潜伏性感染，不是裂解式感染，抗病毒治疗无效NK/T细胞淋巴瘤NK/T细胞淋巴瘤亚型分布NK/T细胞淋巴瘤占到所有PTCL的10.4%JClinOncol,2008,26(25):4124-30NK/T细胞淋巴瘤特征分为鼻型(68%)和非鼻型(26%),其他为侵袭型（6%）病理表现：形态多样，表现为血管中心性、大量坏死和血管浸润表型：大部分为NK细胞（EBV+，CD56+）鼻型与非鼻型NK/T细胞淋巴瘤鼻型非鼻型侵犯部位上呼吸皮肤、睾丸、胃肠道疾病晚期27%68%肿块>5cm12%68%超过2个鼻外病灶16%55%LD...
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