Tse Eric, Liang Raymond H S
University of Hong Kong, Queen Mary Hospital, Pokfulam, Hong Kong.
Clin Lymphoma. 2004 Dec;5(3):197-201. doi: 10.3816/clm.2004.n.027.
Lymphoid neoplasms that are derived from natural killer (NK) cells are uncommon but distinct clinicopathologic disease entities. Three types have been recognized and categorized in the latest World Health Organization classification: extranodal NK cell lymphoma, nasal-type; aggressive NK cell leukemia; and blastic NK cell lymphoma. All NK tumor cells express the NK cell marker CD56, but they lack the expression of surface CD3 and the rearrangement of T-cell receptor genes, which distinguish them from T-lymphoid neoplasms. There is also a strong association with the Epstein-Barr virus, except in blastic NK cell lymphoma. Extranodal involvement by the NK cell tumor is common, especially in the nasal cavity, the skin, and the gastrointestinal tract. All 3 NK cell neoplasms are characterized by aggressive clinical course and poor response to treatment. Although the optimal treatment modality remains to be determined, good initial response to combined radiation therapy and chemotherapy has been observed in localized disease. Further studies in the basic biology of the NK cell and the pathology of NK cell neoplasms may shed light on the development of newer and more effective therapy.
源自自然杀伤（NK）细胞的淋巴样肿瘤并不常见，但却是独特的临床病理疾病实体。最新的世界卫生组织分类中已确认并归类出三种类型：结外NK细胞淋巴瘤，鼻型；侵袭性NK细胞白血病；以及母细胞性NK细胞淋巴瘤。所有NK肿瘤细胞均表达NK细胞标志物CD56，但它们缺乏表面CD3的表达以及T细胞受体基因的重排，这使其有别于T淋巴细胞肿瘤。除母细胞性NK细胞淋巴瘤外，还与EB病毒有很强的关联。NK细胞肿瘤常见结外受累，尤其是在鼻腔、皮肤和胃肠道。所有这三种NK细胞肿瘤的特点都是临床病程侵袭性强且对治疗反应不佳。尽管最佳治疗方式仍有待确定，但在局限性疾病中已观察到联合放疗和化疗有良好的初始反应。对NK细胞基础生物学和NK细胞肿瘤病理学的进一步研究可能会为更新、更有效的治疗方法的开发提供线索。
