主要中枢神经系统淋巴瘤
Andrés J M Ferreri1, Teresa Calimeri2, Kate Cwynarski3
1Lymphoma Unit, IRCCS San Raffaele Scientific Institute, Milan, Italy. ferreri.andres@hsr.it.
Nature reviews. Disease primers
|June 15, 2023
概括
主要中枢神经系统淋巴瘤 (PCNSL) 是一种罕见的脑癌. 目前的治疗方法具有挑战性,许多患者经历了复发,这凸显了改善治疗方法的必要性.
科学领域:
- 神经瘤学神经瘤学
- 血液学 血液学 血液学
- 免疫学 免疫学 免疫学
背景情况:
- 主要中枢神经系统淋巴瘤 (PCNSL) 是一种扩散型大B细胞淋巴瘤,只影响大脑,脊髓,瘤和/或眼睛.
- 它的病理生理学涉及免疫球蛋白与中枢神经系统自身蛋白质结合,并改变了信号通路 (B细胞受体,Toll类受体,NF-κB),免疫细胞和细胞因子的贡献.
- 临床表现不同,取决于所涉及的特定中枢神经系统区域.
研究的目的:
- 为了提供一个全面的概述初级中枢神经系统淋巴瘤 (PCNSL).
- 讨论目前对PCNSL病理生理学,临床表现和标准治疗方法的理解.
- 突出治疗方面的挑战,包括反应率,复发和进一步研究的需要.
主要方法:
- 审查关于初级中枢神经系统淋巴瘤 (PCNSL) 的现有文献.
- 分析当前的标准护理方案,包括化疗,干细胞移植和放射治疗.
- 讨论影响不同患者群体治疗决策的因素.
主要成果:
- 标准治疗包括基于甲基的多化学疗法,其次是自身干细胞移植或放射治疗.
- 很大一部分患者 (15-25%) 对化疗没有反应,25-50%的患者在初始反应后复发.
- 老年患者的复发率更高,复发后的预后通常不好,无论年龄如何.
结论:
- 尽管目前有治疗方法,但由于治疗耐药性和高复发率,PCNSL存在重大挑战.
- 进一步的研究对于识别诊断生物标志物,开发更有效,更少的神经毒性疗法以及探索免疫疗法和采用细胞疗法等新型治疗策略至关重要.
- 改善药物透到中枢神经系统 (CNS) 仍然是治疗进步的关键领域.
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