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.
Advances in diagnosis,molecular pathogenesis and treatment of primary central nervous system lymphoma
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.More-over,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 thera-py,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 lymphomalenalidomideBTK inhibitorsCART therapy
NETL
NSTL
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