Hasserjian Robert P, Harris Nancy Lee
Department of Pathology, Massachusetts General Hospital and Harvard Medical School, Boston, MA 02114, USA.
Am J Clin Pathol. 2007 Jun;127(6):860-8. doi: 10.1309/2F39NX1AL3L54WU8.
Natural killer (NK) cells are lymphocytes that have some phenotypic and functional similarities to cytotoxic T cells but do not express the T-cell receptor complex. NK-cell malignancies may be localized or disseminated at initial examination, and most behave aggressively. The variable presentation of NK-cell lymphomas and leukemias suggests that they represent a spectrum of disease, with Epstein-Barr virus (EBV) implicated in the pathogenesis of most cases. Using cases presented in Session 10 of the 2005 Society for Hematopathology/European Association for Haematopathology Workshop on T-cell and NK-cell malignancies, we discuss outstanding issues in the classification and diagnosis of NK-cell malignancies. These difficulties are related to unusual sites of manifestation, atypical immunophenotypic features, and EBV+ T-cell tumors that resemble classical extranodal NK/T-cell lymphoma, nasal-type (EN-NK/T-NT). Although some of these cases can be grouped into ENNK/T-NT, classification of tumors that have atypical or discordant features will remain controversial, particularly when EBV is absent.
自然杀伤（NK）细胞是一种淋巴细胞，在表型和功能上与细胞毒性T细胞有一些相似之处，但不表达T细胞受体复合物。NK细胞恶性肿瘤在初次检查时可能局限或播散，大多数表现为侵袭性。NK细胞淋巴瘤和白血病的表现各异，提示它们代表了一系列疾病，其中大多数病例的发病机制与爱泼斯坦-巴尔病毒（EBV）有关。利用2005年血液病理学学会/欧洲血液病理学协会T细胞和NK细胞恶性肿瘤研讨会上第10场会议中展示的病例，我们讨论了NK细胞恶性肿瘤分类和诊断中的突出问题。这些困难与不寻常的表现部位、非典型免疫表型特征以及类似于经典结外NK/T细胞淋巴瘤鼻型（EN-NK/T-NT）的EBV阳性T细胞肿瘤有关。尽管其中一些病例可归类为ENNK/T-NT，但具有非典型或不一致特征的肿瘤分类仍存在争议，尤其是在EBV缺失的情况下。
