Objective: To retrospectively analyze the clinical and pathologic characteristics, response to treatment, survival, and prognosis of patients with primary large B-cell lymphoma of the central nervous system (PCNSLBCL) . Methods: Clinical and pathologic data of 70 patients with PCNSLBCL admitted to Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of Medicine from December 2010 to November 2022 were collected for retrospective analysis. Survival analysis was performed using the Kaplan-Meier method and log-rank test, and prognosis analysis was conducted using the Cox proportional hazards model. Results: Among 70 patients with PCNSLBCL, complete remission (CRs) were achieved in 49 (70.0% ) and partial remission in 4 (5.7% ) after the first-line induction therapy; the overall remission rate was 75.7%. The 2-year progression-free survival (PFS) rate was 55.8% and the median progression-free survival (mPFS) time was 35.9 months, whereas the 2-year overall survival (OS) rate was 79.1% with a median OS time not reached. After CR induced by first-line therapy, cumulative incidence of relapse (CIR) was lower in patients who had received auto-HSCT than in those who had not received consolidation therapy (P=0.032), whose 2-year PFS rate was 54.4% and mPFS time was 35.9 months; comparatively, the 2-year PFS rate in patients having received oral maintenance of small molecule drugs reached 84.4% with a mPFS time of 79.5 months (P=0.038). Multivariant analysis demonstrated that Class 3 in the Memorial Sloan-Kettering Cancer Center (MSKCC) prognostic model is an independent adverse prognostic factor of OS in patients with PCNSLBCL (HR=3.127, 95% CI 1.057-9.253, P=0.039) . Conclusions: In patients with PCNSLBCL achieving CR after the first-line induction therapy, auto-HSCT as consolidation therapy would lead to a decreased CIR, and PFS time could be prolonged by oral maintenance of small molecule drugs. Class 3 MSKCC prognostic model is independently associated with poorer OS.
目的： 回顾性分析原发中枢神经系统大B细胞淋巴瘤（PCNSLBCL）患者的临床、病理特征、疗效、生存和预后情况。 方法： 回顾性分析上海交通大学医学院附属瑞金医院自2010年10月至2022年11月收治的70例PCNSLBCL患者的临床和病理资料，采用Kaplan-Meier法及Log-rank检验进行生存分析以及Cox比例风险模型进行预后分析。 结果： 70例PCNSLBCL患者接受一线诱导治疗后49例（70.0%）评价为完全缓解（CR），4例（5.7%）评价为部分缓解，客观缓解率为75.7%。2年无进展生存（PFS）率55.8%，中位PFS期为35.9个月；2年总生存（OS）率79.1%，中位OS期未达到。一线诱导CR后，接受auto-HSCT的患者累积复发率（CIR）低于未接受任何巩固治疗的患者（P＝0.032）；口服小分子药物维持的患者2年PFS率为84.4%，中位PFS期为79.5个月，无巩固治疗患者2年PFS率为54.4%，中位PFS期为35.9个月，差异有统计学意义（P＝0.038）。多因素分析显示纪念斯隆-凯特琳肿瘤中心（MSKCC）预后评分3类是影响PCNSLBCL患者OS的独立预后不良因素（HR＝3.127，95% CI 1.057～9.253，P＝0.039）。 结论： 一线诱导治疗CR的PCNSLBCL患者接受auto-HSCT巩固治疗可降低CIR，口服小分子药物维持治疗可延长PFS期。MSKCC预后评分3类与PCNSLBCL患者较差的OS相关。.
Keywords: Bruton tyrosine kinase; Central nervous system; Lymphoma, large B-cell, diffuse; Mutation; Prognosis.