探讨利妥昔单抗时代原发性结外弥漫性大B细胞淋巴瘤（DLBCL）患者的临床特征和预后因素。病例资料完整且一线接受利妥昔单抗、环磷酰胺、表柔比星治疗的初诊DLBCL患者的连续数据回顾性分析2013年1月至2023年11月内蒙古医科大学附属医院收治的长春新碱、强的松（R-CHOP）或R-CHOP治疗情况。分析结外DLBCL的临床、分子免疫学特征及预后，采用Logistic回归模型分析患者预后的影响因素。共纳入237例患者，其中54.4%（129例）为原发结外来源的DLBCL ，最常见的结外部位如下：胃（19.4%）、结肠（14.7%）、扁桃体（12.4%）、皮肤/肌肉（9.3%）、中央（7.7%）、鼻/鼻咽（6.2%） 、骨髓（5.4%）、睾丸（4.7%）。骨髓、中枢、肝脏、胃肠道或肺部起源的结外DLBCL患者的3年PFS和OS显着低于其他非特殊部位起源的结外DLBCL患者，差异有统计学意义。 PFS：65.2% vs 76.7%，P = 0.008；OS：82.6% vs 88.3%，P = 0.04）。多因素分析显示影响OS的预后因素包括NCCN-IPI评分>3（OR : 0.142, 95%CI : 0.041-0.495, P =0.002）、非生发中心来源（OR : 2.675，95%CI :1.069- 6.694，P =0.036) 和 DEL 患者 (OR : 0.327, 95%CI : 0.129-0.830, P =0.019)。 NCCN-IPI 评分 >3 是 PFS 的唯一独立不良预后因素（OR：0.235，95%CI：0.116-0.474，P < 0.001）。 原发性结外来源的 DLBCL 患者更常见于胃肠道受累，总体而言预后比淋巴结起源的患者差。 NCCN-IPI评分是预测原发性结外弥漫性大B细胞淋巴瘤患者总生存期和无进展生存期的重要独立不良预后因素。

To investigate the clinical features and prognostic factors of patients with primary extranodal diffuse large B-cell lymphoma (DLBCL) in the rituximab era.The continuous data of newly diagnosed DLBCL patients with complete case data and first-line treated with rituximab, cyclophosphamide, epirubicin, vincristine, prednisone (R-CHOP) or R-CHOP treatment admitted to the Affiliated Hospital of Inner Mongolia Medical University from January 2013 to November 2023 were retrospectively analyzed. The clinical and molecular immunological features and prognosis of extranodal DLBCL were analyzed, Logistics regression model was used to analyzed the influencing factors of patients prognosis.A total of 237 patients were enrolled, of which 54.4% (129 cases) were primary extranodal sources of DLBCL, and the most common extranodal sites were as follows: stomach (19.4%), colon (14.7%), tonsils (12.4%), skin/muscle (9.3%), central (7.7%), nasal/nasopharynx (6.2%), bone marrow (5.4%), testes (4.7%). The 3-year PFS and OS of DLBCL patients with extranodal involvement of bone marrow, central, liver, gastrointestinal or pulmonary origin were significantly lower than those of other patients with extranodal DLBCL of non-special site origin, and the difference was statistically significant (PFS: 65.2% vs 76.7%, P =0.008; OS: 82.6% vs 88.3%, P =0.04). Multivariate analysis showed that the prognostic factors affecting OS included NCCN-IPI score >3 (OR : 0.142, 95%CI : 0.041-0.495, P =0.002), non-germinal center source (OR : 2.675，95%CI :1.069-6.694，P =0.036), and DEL patients (OR : 0.327, 95%CI : 0.129-0.830, P =0.019). An NCCN-IPI score >3 was the only independent adverse prognostic factor for PFS (OR : 0.235, 95%CI : 0.116-0.474, P < 0.001).Patients with primary extranodal source DLBCL are more common in gastrointestinal involvement, and the overall prognosis is worse than that of patients with lymph node origin. NCCN-IPI score is an important independent adverse prognostic factor for predicting overall survival and progression-free survival in patients with primary extranodal diffuse large B-cell lymphoma.