目的：分析原发性和继发性胰腺弥漫大B细胞淋巴瘤(DLBCL)的临床特征和预后。方法：分析2003年4月至2020年6月在上海交通大学医学院附属瑞金医院接受治疗的胰腺DLBCL患者的临床数据。采用定向测序（55个淋巴瘤相关基因）评估基因突变谱。使用单因素和多因素Cox回归模型评估总生存期(OS)和无进展生存期(PFS)的预后因素。结果：总共纳入80名患者；12名患者为原发性胰腺DLBCL（PPDLBCL），68名患者为继发性胰腺DLBCL（SPDLBCL）。与PPDLBCL患者相比，SPDLBCL患者受影响的淋巴结外部位更多（P<0.001）且IPI评分更高（P=0.013）。两组之间的OS(P=0.120)和PFS（P=0.067）没有显着差异。多因素分析表明，IPI中高危/高危（P=0.025）和双表达（DE）（P=0.017）是胰腺DLBCL患者OS的独立不良预后因素。 IPI中高危/高危（P=0.021）是胰腺DLBCL患者PFS的独立不良预后因素。对29名患者进行的定向测序显示，胰腺DLBCL患者的PIM1、SGK1、BTG2、FAS、MYC和MYD88的突变频率均>20%。SPDLBCL患者中PIM1(P=0.006 for OS，P=0.032 for PFS) 和MYD88 (P=0.001 for OS，P=0.017 for PFS) 突变与不良OS和PFS相关。结论：PPDLBCL患者和SPDLBCL患者之间的OS和PFS没有显着差异。IPI中高危/高危和DE是胰腺DLBCL的不良预后因素。 PIM1、SGK1、BTG2、FAS、MYC和MYD88是胰腺DLBCL的常见突变。 PIM1和MYD88突变预示着更差的预后。

Objective: To analyze the clinical characteristics and prognosis of primary and secondary pancreatic diffuse large B-cell lymphoma (DLBCL) . Methods: Clinical data of patients with pancreatic DLBCL admitted at Shanghai Rui Jin Hospital affiliated with Shanghai Jiao Tong University School of Medicine from April 2003 to June 2020 were analyzed. Gene mutation profiles were evaluated by targeted sequencing (55 lymphoma-related genes). Univariate and multivariate Cox regression models were used to evaluate the prognostic factors of overall survival (OS) and progression-free survival (PFS) . Results: Overall, 80 patients were included; 12 patients had primary pancreatic DLBCL (PPDLBCL), and 68 patients had secondary pancreatic DLBCL (SPDLBCL). Compared with those with PPDLBCL, patients with SPDLBCL had a higher number of affected extranodal sites (P<0.001) and had higher IPI scores (P=0.013). There was no significant difference in the OS (P=0.120) and PFS (P=0.067) between the two groups. Multivariate analysis indicated that IPI intermediate-high/high risk (P=0.025) and double expressor (DE) (P=0.017) were independent adverse prognostic factors of OS in patients with pancreatic DLBCL. IPI intermediate-high/high risk (P=0.021) was an independent adverse prognostic factor of PFS in patients with pancreatic DLBCL. Targeted sequencing of 29 patients showed that the mutation frequency of PIM1, SGK1, BTG2, FAS, MYC, and MYD88 in patients with pancreatic DLBCL were all >20%. PIM1 (P=0.006 for OS, P=0.032 for PFS) and MYD88 (P=0.001 for OS, P=0.017 for PFS) mutations were associated with poor OS and PFS in patients with SPDLBCL. Conclusion: There was no significant difference in the OS and PFS between patients with PPDLBCL and those with SPDLBCL. IPI intermediate-high/high risk and DE were adverse prognostic factors of pancreatic DLBCL. PIM1, SGK1, BTG2, FAS, MYC, and MYD88 were common mutations in pancreatic DLBCL. PIM1 and MYD88 mutations indicated worse prognosis.