Huo Y J, Zhang M C, Shi Q, Qin W, Shi Z Y, Wang L, Cheng S, Xu P P, Zhao W L
State Key Laboratory of Medical Genomics, Shanghai Institute of Hematology, Shanghai Rui Jin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China.
Zhonghua Xue Ye Xue Za Zhi. 2023 Jan 14;44(1):55-61. doi: 10.3760/cma.j.issn.0253-2727.2023.01.010.
To analyze the clinical characteristics and prognosis of primary and secondary pancreatic diffuse large B-cell lymphoma (DLBCL) . 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) . 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 (<0.001) and had higher IPI scores (=0.013). There was no significant difference in the OS (=0.120) and PFS (=0.067) between the two groups. Multivariate analysis indicated that IPI intermediate-high/high risk (=0.025) and double expressor (DE) (=0.017) were independent adverse prognostic factors of OS in patients with pancreatic DLBCL. IPI intermediate-high/high risk (=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 (=0.006 for OS, =0.032 for PFS) and MYD88 (=0.001 for OS, =0.017 for PFS) mutations were associated with poor OS and PFS in patients with SPDLBCL. 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.
分析原发性和继发性胰腺弥漫性大B细胞淋巴瘤（DLBCL）的临床特征及预后。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收治的胰腺DLBCL患者的临床资料。通过靶向测序（55个淋巴瘤相关基因）评估基因突变谱。采用单因素和多因素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。总体而言，纳入80例患者；12例为原发性胰腺DLBCL（PPDLBCL），68例为继发性胰腺DLBCL（SPDLBCL）。与PPDLBCL患者相比，SPDLBCL患者的结外受累部位数量更多（<0.001），国际预后指数（IPI）评分更高（=0.013）。两组的OS（=0.120）和PFS（=0.067）无显著差异。多因素分析表明，IPI中高/高危（=0.025）和双表达（DE）（=0.017）是胰腺DLBCL患者OS的独立不良预后因素。IPI中高/高危（=0.021）是胰腺DLBCL患者PFS的独立不良预后因素。对29例患者的靶向测序显示，胰腺DLBCL患者中PIM1、SGK1、BTG2、FAS、MYC和MYD88的突变频率均>20%。PIM1（OS为=0.006，PFS为=0.032）和MYD88（OS为=0.001，PFS为=0.017）突变与SPDLBCL患者的不良OS和PFS相关。PPDLBCL患者和SPDLBCL患者的OS和PFS无显著差异。IPI中高/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中的常见突变。PIM1和MYD88突变提示预后较差。
