Tao Kaiyan, Wang Xuefeng, Tian Xin
Department of Neurology, The First Affiliated Hospital of Chongqing Medical University, Chongqing Key Laboratory of Neurology, Chongqing, China.
Front Oncol. 2021 Apr 29;11:649789. doi: 10.3389/fonc.2021.649789. eCollection 2021.
Primary central nervous system lymphoma is an invasive malignant lymphoma confined to the central nervous system. Although patients undergoing first-line treatment can achieve complete response, most of them still relapse within two years. Relapsed lymphoma is derived from occult lymphoma cells, and B cell receptor pathway activation and immune escape are the key mechanisms for the pathogenesis of PCNSL. Most relapses are in the central nervous system, a small number of relapses are isolated systemic relapses, and clinical symptoms occur early and vary. Current treatments for relapse include high-dose methotrexate rechallenge and other regimens of chemotherapy, whole-brain radiation therapy, hematopoietic stem-cell transplantation, targeted therapy and immunotherapy, which have become promising treatments. The overall prognosis of relapsed PCNSL is very poor, although it is affected by many factors. This article summarizes the mechanisms, related factors, clinical features, follow-up, treatment and prognosis of relapsed primary central nervous system lymphoma.
原发性中枢神经系统淋巴瘤是一种局限于中枢神经系统的侵袭性恶性淋巴瘤。尽管接受一线治疗的患者可实现完全缓解，但大多数患者仍会在两年内复发。复发性淋巴瘤源自隐匿的淋巴瘤细胞，B细胞受体途径激活和免疫逃逸是原发性中枢神经系统淋巴瘤发病机制的关键环节。多数复发发生在中枢神经系统，少数为孤立性全身复发，临床症状出现较早且多样。目前针对复发的治疗方法包括大剂量甲氨蝶呤再挑战及其他化疗方案、全脑放射治疗、造血干细胞移植、靶向治疗和免疫治疗，这些已成为有前景的治疗手段。尽管复发性原发性中枢神经系统淋巴瘤的总体预后受多种因素影响，但仍然很差。本文总结了复发性原发性中枢神经系统淋巴瘤的发病机制、相关因素、临床特征、随访、治疗及预后情况。
