EB 病毒相关自然杀伤 (NK) 和 T 细胞淋巴瘤 (EBV NK/T 细胞淋巴瘤) 是一种主要影响儿童和年轻人的严重疾病，通常导致预后不良。迄今为止，尚未就既定治疗策略达成共识。本研究旨在评估 mSMILE（改良类固醇、甲氨蝶呤、异环磷酰胺、L-天冬酰胺酶和依托泊苷）化疗方案治疗 EBV NK/T 细胞淋巴瘤的有效性和安全性，并为潜在的治疗结果提供见解。我们对2017年7月至2022年1月在南京医科大学儿童医院治疗的EBV NK/T细胞淋巴瘤患者的临床资料和治疗结果进行了回顾性分析。这些患者至少接受了两个周期的mSMILE化疗，每个周期用单剂量的培门冬酰胺酶替代 7 剂量的 L-天冬酰胺酶。该研究纳入了 8 名患者：一名患有结外 NK/T 细胞淋巴瘤，一名患有原发性结节 NK/T 细胞淋巴瘤，六名患有结节外 NK/T 细胞淋巴瘤。儿童系统性 EBV NK/T 细胞淋巴瘤。结果显示，5名患者实现完全缓解，2名患者实现部分缓解，1名患者病情进展，完全缓解率为62.5%，总有效率为87.5%。 3年总生存率（OS）和无事件生存率（EFS）分别为87.5%和75%。与化疗相关的最常见不良反应是 III 期至 IV 期的血液学毒性。非血液学不良反应主要包括肝功能受损、感染和口腔粘膜炎，这些不良反应可通过积极抗感染治疗得到解决。根据我们的临床经验，mSMILE 似乎是 EBV NK/T 细胞淋巴瘤安全有效的治疗选择，值得在后期临床试验中进一步研究。2024 转化儿科。版权所有。

The Epstein-Barr virus-associated natural killer (NK) and T-cell lymphoma (EBV + NK/T cell lymphoma) is a severe illness mainly affecting children and young adults, often resulting in a poor prognosis. To date, there is no consensus on an established treatment strategy. This study aims to evaluate the efficacy and safety of the mSMILE (modified steroid, methotrexate, ifosfamide, L-asparaginase, and etoposide) chemotherapy regimen in treating EBV+ NK/T-cell lymphoma and to provide insights into potential treatment outcomes.In this study, we conducted a retrospective analysis of the clinical data and treatment outcomes for patients with EBV + NK/T cell lymphoma treated at Children's Hospital of Nanjing Medical University between July 2017 and January 2022. These patients received at least two cycles of the mSMILE chemotherapy, in which a single dose of pegaspargase was substituted for 7 doses of L-asparaginase per cycle.Eight patients were included in the study: one with extranodal NK/T-cell lymphoma, one with primary nodal NK/T-cell lymphoma, and six with Systemic EBV+ NK/T cell lymphoma of childhood. The results showed that five patients achieved complete remission, two achieved partial remission, and one showed progressive disease, resulting in a complete remission rate of 62.5% and an overall response rate of 87.5%. The 3-year overall survival (OS) and event-free survival (EFS) rates were 87.5% and 75%, respectively. The most common adverse reactions associated with chemotherapy were hematologic toxicities of stages III to IV. Nonhematologic adverse reactions mainly included impaired liver function, infections, and oral mucositis, which were resolved with aggressive anti-infective therapy.Based on our clinical experience, the mSMILE appears to be a safe and effective treatment option for EBV + NK/T-cell lymphoma, meriting further investigation in late-phase clinical trials.2024 Translational Pediatrics. All rights reserved.