摘要
原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma,PCNSL)是一种罕见的侵袭性结外非霍奇金淋巴瘤(non-Hodgkin lymphoma,NHL),主要累及脑、脊髓、软脑膜或玻璃体视网膜间隙,无系统受累证据。PCNSL约占中枢神经系统肿瘤的4%,占所有NHL的4%~6%。与体部淋巴瘤相比,PCNSL的预后较差,五年生存率约为38.3%,十年生存率约30.5%。目前,PCNSL的最佳治疗方案尚未确定,以大剂量甲氨蝶呤(high-dose methotrexate,HD-MTX)为基础的联合化疗仍是推荐的一线治疗方法,常见的联合用药包括阿糖胞苷、利妥昔单抗、替莫唑胺等。随着对PCNSL分子机制及基因表型的深入探索,一些新药如BTK抑制剂(Bruton's tyrosine kinase inhibitor,BTKi)、免疫检查点抑制剂、核输出蛋白1(exportin 1,XPO-1)抑制剂等在淋巴瘤治疗领域令人瞩目的研究进展为PCNSL的治疗带来曙光,本文将对PCNSL的治疗现状与最新进展进行阐述。
Primary central nervous system lymphoma(PCNSL)is a rare invasive non Hodgkin lymphoma(NHL)outside the node,mainly involving the brain,spinal cord,pia mater or vitreoretinal space,without evidence of system involvement,accounting for about 4%of central nervous system tumors and 4%-6%of all NHLs.Compared with body lymphoma,PCNSL has a poor prognosis,with a 5-year survival rate of 38.3%and a 10-year survival rate of 30.5%.At present,the best treatment plan for PCNSL has not been determined.Combined chemotherapy based on high-dose methotrexate(HD-MTX)is still the recommended first-line treatment method.Common combined drugs include cytarabine,rituximab,temozolomide,etc.With the in-depth exploration of the molecular mechanism and gene phenotype of PCNSL,the remarkable research progress of some new drugs in the field of lymphoma treatment,such as BTK inhibitors,immunocheckpoint inhibitors,XOP-1 inhibitors,has brought new opportunities for the treatment of PCNSL.This article will describe the current situation and the latest progress in the treatment of PCNSL.
作者
尹硕
郑晓红
张维春柏
李文斌
陈峰
YIN Shuo;ZHENG Xiaohong;ZHANG Weichunbai;LI Wenbin;CHEN Feng(Department of Neuro-oncology,Cancer Center,Beijing Tiantan Hospital,Beijing 100070,China)
出处
《山东第一医科大学（山东省医学科学院）学报》
CAS
2023年第2期86-92,共7页
Journal of Shandong First Medical University ＆ Shandong Academy of Medical Sciences
关键词
原发性中枢神经系统淋巴瘤
诱导治疗
巩固治疗
新型治疗药物
primary central nervous system lymphoma
induction therapy
consolidation therapy
new therapeutic drugs
