Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China.
Chin Med J (Engl). 2020 Jun 20;133(12):1462-1469. doi: 10.1097/CM9.0000000000000844.
Primary central nervous system lymphoma (PCNSL) is a rare group of extra-nodal non-Hodgkin lymphoma which is confined to the central nervous system or eyes. This article aims to present a brief profile of PCNSL diagnosis and treatment in immunocompetent patients. The authors retrieved information from the PubMed database up to September 2019. The annual incidence of PCNSL increased over the last four decades. The prognosis of PCNSL has improved mainly due to the introduction and wide-spread use of high-dose methotrexate, which is now the backbone of all first-line treatment polychemotherapy regimens. Gene expression profiling and next-generation sequencing analyses have revealed mutations that induce activation of nuclear factor-κB, B cell antigen receptor, and Janus kinases/signal transducer and activator of transcription proteins signal pathways. Some novel agents are investigated in the treatment of relapsed PCNSL including immunotherapy and targeted therapy. In particular, lenalidomide and ibrutinib have demonstrated durable efficiency. Treatment of PCNSL has evolved in the last 40 years and survival outcomes have improved in most patient groups, but there is still room to improve outcome by optimizing current chemotherapy and novel agents.
原发性中枢神经系统淋巴瘤（PCNSL）是一种罕见的结外非霍奇金淋巴瘤，局限于中枢神经系统或眼部。本文旨在简述免疫功能正常患者 PCNSL 的诊断和治疗。作者检索了截至 2019 年 9 月的 PubMed 数据库。在过去的四十年中，PCNSL 的发病率呈上升趋势。PCNSL 的预后得到改善，主要得益于大剂量甲氨蝶呤的应用及其在所有一线治疗联合化疗方案中的广泛应用。基因表达谱和下一代测序分析揭示了诱导核因子-κB、B 细胞抗原受体和 Janus 激酶/信号转导和转录激活蛋白信号通路激活的突变。一些新型药物正在研究用于治疗复发性 PCNSL，包括免疫疗法和靶向治疗。特别是来那度胺和伊布替尼显示出持久的疗效。PCNSL 的治疗在过去 40 年中不断发展，大多数患者群体的生存结果得到改善，但通过优化现有化疗药物和新型药物，仍有改善预后的空间。
