原发性中枢神经系统弥漫性大B细胞淋巴瘤诊疗进展

doi:10.13438/j.cnki.jdzk.2021.06.009

摘要/Abstract

摘要：为进一步提高对原发性中枢神经系统弥漫性大B细胞淋巴瘤（PCNS-DLBCL）的认识，应用PubMed，CNKI等数据库检索“原发性中枢神经系统淋巴瘤诊断、治疗”的相关文献，进而对PCNS-DLBCL的发病机制、临床表现、诊断方法以及治疗措施进行综述.PCNS-DLBCL是结外非霍奇金淋巴瘤的亚型，会累及中枢神经系统的任何部位，其发病机制包括EB病毒的存在，PD-L1和PD-L2的表达增加，MYD88基因突变和CD79B基因突变等；临床表现缺乏特异性，恶性程度高，易复发，预后差；诊断方法包括影像学检查、脑脊液检查、免疫组化及组织病理学检查，病理学检查是确诊的金标准；PCNS-DLBCL治疗措施包括放疗、化疗、自体干细胞移植以及联合放化疗等，目前以施用大剂量甲氨蝶呤为基础的诱导化疗为最佳治疗方案.

关键词： 弥漫性大B细胞淋巴瘤, 中枢神经系统, 诊断方法, 治疗措施, 甲氨蝶呤

Abstract: In order to further improve the understanding of primary central nervous system diffuse large B-cell lymphoma (PCNS-DLBCL), PubMed, CNKI and other databases were used to search related literature on the diagnosis and treatment of primary central nervous system lymphoma, and then The pathogenesis, clinical manifestations, diagnostic methods and treatment measures of PCNS-DLBCL are reviewed. PCNS-DLBCL is a subtype of extranodal non-Hodgkin’s lymphoma that can affect any part of the central nervous system. Its pathogenesis includes the presence of Epstein-Barr virus, increased expression of PD-L1 and PD-L2, MYD88 gene mutation and CD79B Gene mutations, etc.; clinical manifestations lack specificity, high degree of malignancy, easy to relapse, and poor prognosis; diagnostic methods include imaging examination, cerebrospinal fluid examination, immunohistochemistry and histopathological examination, pathological examination is the gold standard for diagnosis; PCNS-DLBCL treatment measures include radiotherapy, chemotherapy, autologous stem cell transplantation, and combined radiotherapy and chemotherapy. At present, induction chemotherapy based on high-dose methotrexate is the best treatment plan.

Key words: diffuse large B-cell lymphoma, central nervous system, diagnostic methods, treatment measures, methotrexate

张汝江, 张运周, 高岱佺. 原发性中枢神经系统弥漫性大B细胞淋巴瘤诊疗进展[J]. 吉首大学学报（自然科学版）, 2021, 42(6): 54-61.

ZHANG Rujiang, ZHANG Yunzhou, GAO Daiquan. Progress in the Diagnosis and Treatment of Primary Central Nervous System Diffuse Large B-Cell Lymphoma[J]. Journal of Jishou University(Natural Sciences Edition), 2021, 42(6): 54-61.

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