Objective: To explore the clinical features and prognosis of patients with primary central nervous system lymphoma（PCNSL）.
Methods: A retrospective analysis was performed on the relationship between clinical features, treatment regimen and prognosis in 46 newly diagnosed patients with primary central nervous system lymphoma who were diagnosed and treated in The Second Hospital of Lanzhou University from January 2015 to September 2022. Fisher's exact probability method was used to analyze the differences in clinical data of different subgroups. Kaplan-Meier survival curve was used to analyze the overall survival rate and progression-free survival rate of patients with different treatments, and the factors influencing survival were analyzed.
Results: Among 46 patients with PCNSL, which pathological type were diffuse large B-cell lymphoma（DLBCL）. There were 26(56.5%) cases of male and 20(43.5%) of female, with a median age of 54(17-71) years. In Hans subtypes, 14 cases (30.4%) of GCB subtype, 32 cases (69.6%) of non-GCB subtype. 32 cases (69.6%) of Ki-67≥80%. Among 36 patients who completed at least 2 cycles of treatment with follow-up data, the efficacy evaluation was as follows: overall response rate(ORR) was 63.9%, complete response(CR) rate was 47.2%, 17 cases of CR, 6 cases of PR. The 1-year progression-free survival rate and 1-year overall survival rate was 73.6% and 84.9%, respectively. The 2-year progression-free survival rate and 2-year overall survival rate was 52.2% and 68.9%, respectively. The ORR and CR rate of 17 patients treated with RMT regimen was 76.5% and 52.9% (9 cases CR and 4 cases PR), respectively. Univariate analysis of 3 groups of patients treated with RMT regimen, RM-BTKi regimen, and RM-TT regimen as first-line treament showed that deep brain infiltration was associated with adverse PFS(P =0.032), and treatment regimen was associated with adverse OS in PCNSL patients（P =0.025）.
Conclusion: Different treatment modalities were independent prognosis predictors for OS, the deep brain infiltration of PCNSL is a poor predictive factor for PFS. Patients with relapse/refractory (R/R) PCNSL have a longer overall survival time because to the novel medication BTKi. They have strong toleration and therapeutic potential as a first-line therapy for high-risk patients.
题目: 原发中枢神经系统淋巴瘤患者的临床特征及预后分析.
目的: 探讨原发中枢神经系统淋巴瘤（PCNSL）患者的临床特征及预后。.
方法: 回顾性分析2015年1月至2022年9月在本院收治的46例新诊断PCNSL患者的临床特征、治疗方案与预后的关系。运用Fisher 确切概率法分析不同亚组临床资料的差异，采用Kaplan-Meier生存曲线分析不同治疗方案患者的总生存率（OS）和无进展生存率（PFS），并分析影响患者生存的影响因素。.
结果: 46例PCNSL患者病理类型均为弥漫大B细胞淋巴瘤，其中男性26例（56.5%），女性20例（43.5%），中位年龄为54（17-71）岁。Hans分型中，GCB亚型14例（30.4%），non-GCB亚型32例（69.6%），Ki-67≥80%者32例（69.6%）。36例完成至少2疗程治疗并有随访资料的患者，2疗程后疗效评估：完全缓解（CR）率为47.2%、总缓解率（ORR）为63.9%，其中达完全缓解17例，部分缓解（PR）6例，1年PFS率与1年OS率分别为73.6%和84.9%，2年PFS率及2年OS率分别为52.2%和68.9%。17例以RMT方案治疗的患者ORR为76.5%（13/17），CR率为52.9%（9/17）。对以RMT（利妥昔单抗/甲氨蝶呤/替莫唑胺）方案、RM-BTKi（利妥昔单抗/甲氨蝶呤/BTK抑制剂）方案与RM-TT（利妥昔单抗/甲氨蝶呤/塞替派）方案作为一线治疗的3组患者进行单因素分析显示，脑深部浸润与 PCNSL患者不良PFS相关（P =0.032），治疗方案与不良OS相关（P =0.025）。.
结论: PCNSL患者脑深部浸润是PFS的不良预后因素，治疗方案对OS有影响。新药BTKi的加入能够延长复发/难治PCNSL患者的总生存期，在高危患者的一线治疗中显示出其良好的耐受性及治疗潜力。.
Keywords: primary central nervous system lymphoma; DLBCL; BTK inhibitor； Thiotepa; prognosis.