原发中枢神经系统淋巴瘤诊治进展

陈弘韵; 张薇

doi:10.13201/j.issn.1004-2806.2024.09.005

原发中枢神经系统淋巴瘤诊治进展

陈弘韵¹,
张薇^1, ,

- 中国医学科学院北京协和医院血液科(北京，100730)

详细信息

作者简介:
张薇，北京协和医院血液内科主任医师，从事血液病专业近三十年。担任中国老年淋巴瘤学会青年委员，中国抗癌协会血液肿瘤专业委员，中国免疫学会血液免疫分会委员，中国抗淋巴瘤联盟常委，中国抗癌协会淋巴瘤整合康复专业副主委，北京医学奖励基金会血液疾病专家委员会秘书

通讯作者: 张薇，E-mail：vv1223@vip.sina.com

中图分类号: R733.4

收稿日期: 2024-05-28

刊出日期: 2024-09-01

Advances in diagnosis, molecular pathogenesis and treatment of primary central nervous system lymphoma

CHEN Hongyun¹,
ZHANG Wei^1, ,

- Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, 100730, China

More Information

Corresponding author: ZHANG Wei, E-mail: vv1223@vip.sina.com

Received Date: 28 May 2024

Publish Date: 01 September 2024

摘要

摘要: 原发中枢神经系统淋巴瘤(primary central nervous system lymphoma，PCNSL)是一类罕见的侵袭性结外非霍奇淋巴瘤，具有独特的临床表现和生物学特征。基于大剂量甲氨蝶呤的系统化疗极大改善了该病的临床结局。然而，仍有部分患者对化疗耐药或最终出现复发。这些复发/难治性PCNSL患者对传统治疗反应欠佳，临床预后极差。因此，探寻新型疗法尤为重要。随着靶向药物、免疫治疗和细胞治疗的进展，如来那度胺、Bruton酪氨酸激酶抑制剂、嵌合抗原受体T细胞治疗越来越多地应用于PCNSL患者的治疗。
- 原发中枢神经系统淋巴瘤 /
- 来那度胺 /
- BTK抑制剂 /
- CAR-T细胞治疗
Abstract: Primary central nervous system lymphoma(PCNSL) is a rare, aggressive extra-nodal non-Hodgkin's lymphoma with unique clinical and biologic characteristics. The application of systemic chemotherapy based on high-dose methotrexate improves the clinical outcome. Nevertheless, a considerable fraction of patients are refractory to chemotherapies or undergo relapses eventually. The outcomes of these patients are dismal. Moreover, the effect of traditional treatment for recurrent/refractory PCNSL patients is not satisfactory. Therefore, it is significant to explore new therapies. With the development of targeted agents, immunotherapies and cell therapy, novel treatments including lenalidomide, Bruton's tyrosine kinase inhibitors, chimeric antigen receptor T-cell therapy are being attempted in PCNSL patients.
- primary central nervous system lymphoma /
- lenalidomide /
- BTK inhibitors /
- CAR-T therapy

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表 1 初治PCNSL一线治疗相关临床研究

研究编号	分期	治疗方案	例数	初步结果
NCT04514393	2期	甲氨蝶呤、伊布替尼、替莫唑胺(MIT)	33	－
ChiCTR1900027811	1期	伊布替尼、利妥昔单抗、甲氨蝶呤(I-MR)	16	总有效率(ORR)为94% 中位PFS、OS未达到预估3年PFS率、OS率分别为88.9%、86.5%
NCT05549284	2期	利妥昔单抗、甲氨蝶呤、奥布替尼(RMO)	36	ORR为100%，CR率为61.8% 完成6个疗程治疗的18例患者ORR为94.4%，CR率为89.89%
NCT04934579	2期	来那度胺、利妥昔单抗、甲氨蝶呤(R2-MTX)	17	ORR为88.2%，CR率为76.5%

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