结外NK/T细胞淋巴瘤，鼻型的诊断与治疗进展

结外NK/T细胞淋巴瘤，鼻型（extranodal natural killer/T-cell lymphoma ，nasal type，ENKL）是非霍奇金淋巴瘤（non-hodgkin lymphoma，NHL ）的一种少见亚型，其侵袭性强且预后较差。ENKL 主要发生于鼻腔，其次是皮肤、胃肠道等。该病以血管的侵犯和组织破坏为主要病理学表现。ENKL 与EBV 的感染密切相关，EBV 水平对其辅助诊断有重要的意义。NK/T细胞表面的特征性标志物和特异性遗传学改变也可以帮助诊断该病。目前对于ENKL 的治疗尚在讨论中，虽然对早期患者采用放疗± 化疗联合治疗，以及对中晚期患者采用以左旋门冬酰胺酶为基础的化疗和造血干细胞移植得到了一定疗效，但仍需进一步的研究探索以形成规范的治疗原则。 

Diagnosis and treatment of extranodal NK/T-cell lymphoma,nasal type

Extranodal natural killer (NK)/T-cell lymphoma, nasal type (ENKL) is an uncommon subtype of non-Hodgkin's lymphoma, which is aggressive and has poor prognosis. ENKL occurs predominantly in the nasal cavity and less frequently in the skin and in the gastrointestinal tract. The pathology of this disease is characterized by vascular invasion and destruction. ENKL is strongly associated with the Epstein–Barr virus, which aids in the diagnosis of ENKL. The characteristic markers on the surface of an NK/T cell and the spe-cific genetic variations are also useful in the diagnosis. At present, the treatment of ENKL is still under discussion. Although radiothera-py combined with chemotherapy on patients with early-stage ENKL and L-ASP-based chemotherapy and hematopoietic stem cell trans-plant on patients with advance-stage ENKL have achieved favorable effects, further studies are still necessary to develop the principles and methods of a standardized treatment.