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 2008 WHO 2008: the mature T-cell and NK-cell neoplasms T-cell prolymphocytic leukemia T-cell large granular lymphocytic leukemia Chronic lymphoproliferative disorder of NK-cells* Aggressive NK cell leukemia Systemic EBV+ T-cell lymphoprolife
2、rative disease of childhood (associated with CAEBV) Hydroa vacciniforme-like lymphoma Adult T-cell leukemia/lymphoma Extranodal NK/T cell lymphoma, nasal type Enteropathy-associated T-cell lymphoma Hepatosplenic T-cell lymphoma Subcutaneous panniculitis-like T-cell lymphoma Mycosis fungoides Szary s
3、yndrome Primary cutaneous CD30+ T-cell lymphoproliferative disorder Lymphomatoid papulosis Primary cutaneous anaplastic large-cell lymphoma Primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma* Primary cutaneous gamma-delta T-cell lymphoma Primary cutaneous small/medium CD4+ T-
4、cell lymphoma* Peripheral T-cell lymphoma, not otherwise specified Angioimmunoblastic T-cell lymphoma Anaplastic large cell lymphoma (ALCL), ALK+ Anaplastic large cell lymphoma (ALCL), ALK* 2001 WHO 2008 WHO Comments Angioimmunoblastic Lymphoma Angioimmunoblastic Lymphoma Definition of origin cell A
5、naplastic Large Cell Lymphoma 2 variants based on ALK (+/-) expression Prognostic importance Unspecified Peripheral T-cell Lymphoma Peripheral T-cell Lymphomas not Otherwise Specified 3 variants: lymphoepitelioid lymphoma, T zone lymphoma (2001 WHO) and follicular lymphoma (2008 WHO) T/NK-cell lymph
6、oma, nasal type T/NK-cell lymphoma, nasal type No changes Entheropathy-associated T-cell lymphoma Entheropathy-associated T-cell lymphomas Two variants: classical and monomorphic types with genetic changes common to both Hepatosplenic T-cell lymphoma Hepatosplenic T-cell lymphoma No changes Subcutan
7、eous panniculitis-like T-cell lymphoma Subcutaneous panniculitis-like T-cell lymphoma Only ab and associated with autoimmune disorder Mycosis fungoides Mycosis fungoides New staging and new information about pathogenesis Szary syndrome Szary syndrome New markers Primary cutaneous anaplastic large ce
8、ll lymphoma Primary cutaneous anaplastic large cell lymphoma Recognition of CD8+ cases Lymphomatoid papulosis Lymphomatoid papulosis Three histological types Primary cutaneous gamma-delta T-cell lymphoma Three histopathologic patterns: epidermotropic, dermic, and subcutaneous subtypes Primary cutane
9、ous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma Provisional entity Primary cutaneous CD4+ small/medium T-cell lymphoma Provisional entity Blastic NK-cell lymphoma Plasmocytoid dendritic cell neoplasm Now it is one of the myeloid neoplasms T-cell prolymphocytic leukemia T-cell prolymphoc
10、ytic leukemia No changes T-cell large granular lymphocytic leukemia T-cell large granular lymphocytic leukemia New etiological features and new markers Chronic lymphoproliferative disorder of NK-cells Provisional entity Aggressive NK-cell leukemia Aggressive NK-cell leukemia No changes Adult T-cell
11、leukemia/lymphoma Adult T-cell leukemia/lymphoma Definition of the regulatory T-cell normal counterpart T 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):4124-30 NK/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+）
