Section of Hematology/Oncology, Department of Medicine, VA Long Beach Healthcare System, Long Beach, CA, USA.
Division of Hematology-Oncology, Department of Medicine, University of California, Irvine, CA, USA.
Leuk Lymphoma. 2023 Dec;64(12):1964-1970. doi: 10.1080/10428194.2023.2245513. Epub 2023 Aug 11.
Mature T-cell and NK-cell lymphomas (MTNKL) are rare and heterogeneous lymphoproliferative disorders with poor clinical outcomes despite novel therapeutic advances. Although infrequent, central nervous system (CNS) involvement by MTNKL is associated with poor outcomes with a median overall survival (OS) of <12 months based on retrospective studies. We performed a retrospective analysis of patients who developed CNS involvement of MTNKL diagnosed at a single center from 1999 through 2020. Twenty-five patients were identified. Characteristics such as a diagnosis of adult T-cell leukemia/lymphoma, extranodal involvement, and poor performance status were associated with a higher risk of CNS involvement ( < 0.01). The median OS after diagnosis with CNS involvement was approximately 1 month (0.03-103.97 months). Patients exposed to novel therapeutics and/or clinical trial enrollment tolerated treatment without safety concerns and appeared to derive reasonable therapeutic benefit. Despite advances in the field, new therapeutic approaches are needed for patients with MTNKL with CNS involvement.
成熟 T 细胞和自然杀伤细胞淋巴瘤（MTNKL）是罕见且异质性的淋巴增生性疾病，尽管有新的治疗进展，但临床结局仍较差。尽管不常见，但基于回顾性研究，MTNKL 中枢神经系统（CNS）受累与预后不良相关，中位总生存期（OS）<12 个月。我们对 1999 年至 2020 年在单一中心诊断为 MTNKL 且发生 CNS 受累的患者进行了回顾性分析。共确定了 25 名患者。诊断为成人 T 细胞白血病/淋巴瘤、结外受累和较差的体能状态等特征与 CNS 受累风险较高相关（<0.01）。诊断为 CNS 受累后的中位 OS 约为 1 个月（0.03-103.97 个月）。接受新型治疗药物和/或临床试验入组的患者耐受治疗，无安全性问题，且似乎获得了合理的治疗获益。尽管该领域取得了进展，但仍需要为患有 CNS 受累的 MTNKL 患者开发新的治疗方法。
