Gill Harinder, Liang Raymond H S, Tse Eric
Division of Haematology, Medical Oncology and Bone Marrow Transplantation, Department of Medicine, University of Hong Kong, Queen Mary Hospital, Hong Kong.
Adv Hematol. 2010;2010:627401. doi: 10.1155/2010/627401. Epub 2010 Dec 30.
The World Health Organization (WHO) classification recognizes 2 main categories of natural killer (NK) cell-derived neoplasms, namely, extranodal NK/T-cell lymphoma, nasal type, and aggressive NK-cell leukaemia. Extranodal nasal NK/T-cell lymphoma is more frequent in the Far East and Latin America. Histopathological and immunophenotypical hallmarks include angiocentricity, angiodestruction, expression of cytoplasmic CD3 epsilon (ε), CD56, and cytotoxic molecules and evidence of Epstein-Barr virus (EBV) infection. Early stage disease, in particular for localized lesion in the nasal region, is treated with chemotherapy and involved-field radiotherapy. On the other hand, multiagent chemotherapy is the mainstay of treatment for advanced or disseminated disease. L-asparaginase-containing regimens have shown promise in treating this condition. The role of autologous hematopoietic stem cell transplantation is yet to be clearly defined. Allogeneic hematopoietic stem cell transplantation, with the putative graft-versus-lymphoma effect, offers a potentially curative option in patients with advanced disease.
世界卫生组织（WHO）分类认可自然杀伤（NK）细胞来源的肿瘤主要有2大类，即结外NK/T细胞淋巴瘤，鼻型，以及侵袭性NK细胞白血病。结外鼻型NK/T细胞淋巴瘤在远东和拉丁美洲更为常见。组织病理学和免疫表型特征包括血管中心性、血管破坏、细胞质CD3ε、CD56和细胞毒性分子的表达以及爱泼斯坦-巴尔病毒（EBV）感染的证据。早期疾病，特别是鼻区局限性病变，采用化疗和受累野放疗。另一方面，多药化疗是晚期或播散性疾病的主要治疗方法。含L-天冬酰胺酶的方案在治疗这种疾病方面已显示出前景。自体造血干细胞移植的作用尚未明确界定。具有假定移植物抗淋巴瘤效应的异基因造血干细胞移植为晚期疾病患者提供了一种潜在的治愈选择。
