Homsy Sylvester, Kamel Ralph, Raden Mark, Skaradinskiy Yevgeniy
Department of Internal Medicine, Staten Island University Hospital-Northwell Health, Staten Island, New York, NY, USA.
Department of Radiology, Staten Island University Hospital-Northwell Health, Staten Island, New York, NY, USA.
Case Rep Hematol. 2021 Jul 24;2021:9972694. doi: 10.1155/2021/9972694. eCollection 2021.
Extranodal natural killer/T-cell lymphoma nasal type (NNKTL) is a type of non-Hodgkin's lymphoma that has been associated with Epstein-Barr virus (EBV). It has an aggressive behavior, known for predilection to metastasize to different organs. Central nervous system (CNS) spread from a primary location has been reported. Different modalities of treatment such as chemotherapy and radiation therapy have been employed in the management of this disease. Severe toxicities of currently available treatment have made clinicians seek more targeted therapies using molecular profiling. We present a 44-year-old Hispanic patient who was diagnosed with an early-stage NNKTL and treated with the modified SMILE regimen for 6 cycles. His EBV DNA PCR turned undetectable and remained so throughout the treatment. He sustained complete right vision loss due to right optic nerve invasion by the tumor, leading to prophylactic intravitreal methotrexate to the contralateral eye. The patient achieved good response with minimal residual disease. He was supposed to start radiation as a sequential therapy. However, the acute development of severe headache and confusion lead to a complete workup showing leptomeningeal spread. He eventually succumbed to the disease.
鼻型结外自然杀伤细胞/T细胞淋巴瘤（NNKTL）是一种与爱泼斯坦-巴尔病毒（EBV）相关的非霍奇金淋巴瘤。它具有侵袭性，以易转移至不同器官而闻名。已有报道其可从原发部位扩散至中枢神经系统（CNS）。化疗和放疗等不同治疗方式已用于该疾病的管理。目前可用治疗的严重毒性促使临床医生寻求更多基于分子谱分析的靶向治疗。我们报告一名44岁的西班牙裔患者，他被诊断为早期NNKTL，并接受改良的SMILE方案治疗6个周期。他的EBV DNA PCR检测不到，且在整个治疗过程中一直如此。由于肿瘤侵犯右侧视神经，他出现了完全性右眼视力丧失，因此对侧眼接受了预防性玻璃体内甲氨蝶呤注射。该患者获得了良好反应，残留疾病极少。他本应开始接受序贯放疗。然而，严重头痛和意识模糊的急性发作导致全面检查显示软脑膜扩散。他最终死于该疾病。
