1、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： WHO 2008WHO 2008: the mature T-cell and NK-cell neoplasmsT-cell prolymphocytic leukemiaT-cell large granular lymphocytic leukemiaChronic lymphoproliferative disorder of NK-cells*Aggressive NK cell leukemiaSystemic EBV+ T-cell lymphoproliferative dis
2、ease of childhood (associated with CAEBV)Hydroa vacciniforme-like lymphomaAdult T-cell leukemia/lymphomaExtranodal NK/T cell lymphoma, nasal typeEnteropathy-associated T-cell lymphomaHepatosplenic T-cell lymphomaSubcutaneous panniculitis-like T-cell lymphomaMycosis fungoidesSzary syndromePrimary cut
3、aneous CD30+ T-cell lymphoproliferative disorderLymphomatoid papulosisPrimary cutaneous anaplastic large-cell lymphomaPrimary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma*Primary cutaneous gamma-delta T-cell lymphomaPrimary cutaneous small/medium CD4+ T-cell lymphoma*Peripheral
4、 T-cell lymphoma, not otherwise specifiedAngioimmunoblastic T-cell lymphomaAnaplastic large cell lymphoma (ALCL), ALK+Anaplastic large cell lymphoma (ALCL), ALK*2001 WHO 2008 WHO CommentsAngioimmunoblastic Lymphoma Angioimmunoblastic Lymphoma Definition of origin cellAnaplastic Large Cell Lymphoma 2
5、 variants based on ALK (+/-) expression Prognostic importanceUnspecified Peripheral T-cell Lymphoma Peripheral T-cell Lymphomas not Otherwise Specified3 variants: lymphoepitelioid lymphoma, T zone lymphoma (2001 WHO) and follicular lymphoma (2008 WHO) T/NK-cell lymphoma, nasal type T/NK-cell lymphom
6、a, nasal type No changesEntheropathy-associated T-cell lymphoma Entheropathy-associated T-cell lymphomas Two variants: classical and monomorphic types with genetic changes common to bothHepatosplenic T-cell lymphoma Hepatosplenic T-cell lymphoma No changesSubcutaneous panniculitis-like T-cell lympho
7、ma Subcutaneous panniculitis-like T-cell lymphoma Only ab and associated with autoimmune disorderMycosis fungoides Mycosis fungoides New staging and new information about pathogenesis Szary syndrome Szary syndrome New markersPrimary cutaneous anaplastic large cell lymphoma Primary cutaneous anaplast
8、ic large cell lymphoma Recognition of CD8+ casesLymphomatoid papulosis Lymphomatoid papulosis Three histological typesPrimary cutaneous gamma-delta T-cell lymphoma Three histopathologic patterns: epidermotropic, dermic, and subcutaneous subtypesPrimary cutaneous CD8+ aggressive epidermotropic cytoto
9、xic T-cell lymphoma Provisional entityPrimary cutaneous CD4+ small/medium T-cell lymphoma Provisional entityBlastic NK-cell lymphoma Plasmocytoid dendritic cell neoplasm Now it is one of the myeloid neoplasmsT-cell prolymphocytic leukemia T-cell prolymphocytic leukemia No changesT-cell large granula
10、r lymphocytic leukemiaT-cell large granular lymphocytic leukemia New etiological features and new markersChronic lymphoproliferative disorder of NK-cells Provisional entityAggressive NK-cell leukemia Aggressive NK-cell leukemia No changesAdult T-cell leukemia/lymphoma Adult T-cell leukemia/lymphoma
11、Definition of the regulatory T-cell normal counterpartT 和 NK 细胞肿瘤分类的主要变化EBV 相关淋巴增殖性疾病J Korean Med Sci. 2008 Apr;23(2):185-92.EBV 相关 T/NK 细胞增殖性疾病J Dermatol. 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%J Clin Oncol, 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+）
