原发性中枢神经系统淋巴瘤诊治现状及进展

Primary central nervous system lymphoma: status and advances in diagnosis, molecular pathogenesis and treatment

原发中枢神经系统淋巴瘤(PCNSL)是罕见结外非霍奇金淋巴瘤亚型,具有独特的临床表现和生物学特性。以大剂量甲氨蝶呤为基础化疗方案改善了PCNSL的临床结局,但仍有过半数患者出现复发或耐药。介绍PCNSL的临床表现及诊治现状,特别是近年来该病在诊断及分子学发病机制的进展、新型药物在复发难治患者中的应用。

Primary central nervous system lymphoma (PCNSL) is a rare group of extra-nodal non-Hodgkin lymphoma with specific biological characteristics and clinical behaviors. The systemic chemotherapy based on highdose methotrexate has improved the clinic outcomes, but risk of relapse and tolerance is still substantial. In this paper,we will review the status of diagnosis and treatment of PCNSL, especially advances in the diagnosis, molecular pathogenesis and novel agents in relapsed/refractory cases.