原发性中枢神经系统淋巴瘤的治疗进展

Advances in the treatment of primary central nervous system lymphoma

原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma,PCNSL)是累及脑脊液、脊髓、眼内结构、颅神经及软脑膜的侵袭性结外非霍奇金淋巴瘤,无中枢神经系统以外部位受累的一种罕见浸润性恶性肿瘤。PCNSL对放化疗敏感,治疗包括以大剂量甲氨蝶呤为基础的诱导缓解治疗,手术和全脑放疗的巩固治疗。近年来出现包括BTK抑制剂、PD-1抑制剂、来那度胺、利妥昔单抗、培美曲塞、CAR-T疗法、核输出抑制剂等新药进入临床试验或使用阶段,并显示出良好的治疗效果。本文对PCNSL患者的传统治疗方法及新的靶向药物治疗进展进行综述。

Primary central nervous system lymphoma(PCNSL)is an aggressive extranodal non-Hodgkin's lymphoma involving cerebrospinal fluid,medulla spinalis,intraocular structures,cranial nerves,and pia.It is an infiltrating malignant tumor with no involvement outside the central nervous system.PCNSL is sensitive to radiotherapy and chemotherapy,and treatment includes high-dose methotrexate-based remission induction,surgery and consolidation therapy with whole-brain radiotherapy.In recent years,some new drugs,including Bruton tyrosine kinase inhibitor,programmed cell death protein-1 inhibitor,lenalidomide,rituximab,pemetrexed,chimeric antigen receptor T-cell immunotherapy and selective inhibitor of nuclear export,have entered clinical trials or use stage and shown good therapeutic effect.This article reviews the traditional treatment programs of PCNSL patients and summarizes the latest progress of treatment.