原发性中枢神经系统淋巴瘤的治疗进展

Advances in the treatment of primary central nervous system lymphoma

原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma,PCNSL)是一种罕见的结外淋巴瘤,其病理类型多为弥漫性大B细胞淋巴瘤,影像学多表现为单发的脑实质深部病变,MRI可有多种强化形态。目前,以大剂量甲氨蝶呤为主的化疗已成为PCNSL的一线治疗。放、化疗的结合可延长患者的生存期,但神经毒性发生率较高。大剂量化疗联合自体干细胞移植对复发/难治性PCNSL有效。替莫唑胺和利妥昔单抗不良反应小、耐受性好,可作为PCNSL治疗的选择。PCNSL的预后受血清LDH浓度、年龄、ECOG评分/KPS评分、脑脊液蛋白浓度、肿瘤定位等多种因素的影响。

Primary central nervous system lymphoma （PCNSL） is a rare extranodal lymphoma. Its pathological type mainly includes dif-fuse large B cell lymphoma. In MRI images, the condition is usually manifested as solitary deep lesions in brain parenchyma, with vari -ous forms of reinforcement. To date, chemotherapy based on high-dose methotrexate administration is the first-line therapy of PCNSL. Combined radiotherapy and chemotherapy can prolong the overall survival of patients. However, this approach simultaneously causes higher incidence of neurotoxicity. High-dose chemotherapy followed by autologous stem cell transplantation is recommended for re-current and refractory PCNSL. Temozolomide and rituximab can be used as treatment options because of their low toxicity and good tolerance. The prognosis of PCNSL depends on several factors, such as serum LDH concentration, age, ECOG /KPS score、cerebro-spi -nal fluid （CSF） protein concentration and tumor location.