Reneau John C, Shindiapina Polina, Braunstein Zachary, Youssef Youssef, Ruiz Miguel, Farid Saira, Hanel Walter, Brammer Jonathan E
Division of Hematology, Department of Internal Medicine, James Comprehensive Cancer Center, The Ohio State University, Columbus, OH 43210, USA.
Department of Internal Medicine, Wexner Medical Center, The Ohio State University, Columbus, OH 43210, USA.
J Clin Med. 2022 May 10;11(10):2699. doi: 10.3390/jcm11102699.
Extranodal natural killer/T(NK/T)-cell lymphoma (ENKTL) is a rare subtype of non-Hodgkin lymphoma that typically presents with an isolated nasal mass, but a sizeable minority present with advanced stage disease and have a significantly poorer prognosis. Those with limited disease are standardly treated with chemotherapy and radiation while those with advanced stage disease are treated with L-asparaginase containing chemotherapy regimens. The addition of modern radiation therapy techniques and the incorporation of L-asparaginase into chemotherapy regimens have significantly improved outcomes in this disease, but relapses and death from relapsed disease remain frequent. Given the high rate of relapse, several novel therapies have been evaluated for the treatment of this disease. In this review, we explore the current standard of care for ENKTL as well as novel therapies that have been evaluated for its treatment and the biologic understanding behind these therapies.
结外自然杀伤/T（NK/T）细胞淋巴瘤（ENKTL）是非霍奇金淋巴瘤的一种罕见亚型，通常表现为孤立的鼻腔肿物，但有相当一部分患者表现为晚期疾病，预后明显较差。局限性疾病患者通常采用化疗和放疗，而晚期疾病患者则采用含L-天冬酰胺酶的化疗方案治疗。现代放疗技术的应用以及L-天冬酰胺酶纳入化疗方案显著改善了该疾病的治疗效果，但复发以及复发导致的死亡仍然很常见。鉴于高复发率，已对几种新型疗法进行了评估以治疗该疾病。在本综述中，我们探讨了ENKTL目前的治疗标准以及已评估用于其治疗的新型疗法及其背后的生物学认识。
