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Objective: To analyze the clinicopathologic characteristics and prognosis of testicular diffuse large B-cell lymphoma (DLBCL) . Methods: A retrospective analysis was performed on 68 patients with testicular DLBCL admitted to Ruijin Hospital affiliated to Shanghai Jiao Tong University School of Medicine from October 2001 to April 2020. The gene mutation profile was evaluated by targeted sequencing (55 lymphoma-related genes) , and prognostic factors were analyzed. Results: A total of 68 patients were included, of whom 45 (66.2% ) had primary testicular DLBCL and 23 (33.8% ) had secondary testicular DLBCL. The proportion of secondary testicular DLBCL patients with Ann Arbor stage Ⅲ-Ⅳ ( P <0.001) , elevated LDH ( P <0.001) , ECOG score ≥ 2 points ( P =0.005) , and IPI score 3-5 points ( P <0.001) is higher than that of primary testicular DLBCL patients. Sixty-two (91% ) patients received rituximab in combination with cyclophosphamide, adriamycin, vincristine, and prednisone (R-CHOP) -based first-line regimen, whereas 54 cases (79% ) underwent orchiectomy prior to chemotherapy. Patients with secondary testicular DLBCL had a lower estimated 5-year progression-free survival (PFS) rate (16.5% vs 68.1% , P <0.001) and 5-year overall survival (OS) rate (63.4% vs 74.9% , P =0.008) than those with primary testicular DLBCL, and their complete remission rate (57% vs 91% , P =0.003) was also lower than that of primary testicular DLBCL. The ECOG scores of ≥2 (PFS: P =0.018; OS: P <0.001) , Ann Arbor stages Ⅲ-Ⅳ (PFS: P <0.001; OS: P =0.018) , increased LDH levels (PFS: P =0.015; OS: P =0.006) , and multiple extra-nodal involvements (PFS: P <0.001; OS: P =0.013) were poor prognostic factors in testicular DLBCL. Targeted sequencing data in 20 patients with testicular DLBCL showed that the mutation frequencies of ≥20% were PIM1 (12 cases, 60% ) , MYD88 (11 cases, 55% ) , CD79B (9 cases, 45% ) , CREBBP (5 cases, 25% ) , KMT2D (5 cases, 25% ) , ATM (4 cases, 20% ) , and BTG2 (4 cases, 20% ) . The frequency of mutations in KMT2D in patients with secondary testicular DLBCL was higher than that in patients with primary testicular DLBCL (66.7% vs 7.1% , P =0.014) and was associated with a lower 5-year PFS rate in patients with testicular DLBCL ( P =0.019) . Conclusion: Patients with secondary testicular DLBCL had worse PFS and OS than those with primary testicular DLBCL. The ECOG scores of ≥2, Ann Arbor stages Ⅲ-Ⅳ, increased LDH levels, and multiple extra-nodal involvements were poor prognostic factors in testicular DLBCL. PIM1, MYD88, CD79B, CREBBP, KMT2D, ATM, and BTG2 were commonly mutated genes in testicular DLBCL, and the prognosis of patients with KMT2D mutations was poor.

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.

Objective: To investigate the efficacy of RCDOP (Rituximab, cyclophosphamide, liposome doxorubicin, vincristine and prednisone) regimen in patients with de novo diffuse large B-cell lymphoma (DLBCL), especially in those patients with multiple extra-nodal involvement or Bulky diseases. Methods: A total of 87 newly diagnosed DLBCL patients who received RCDOP regimen from October 2012 to October 2017 were enrolled into this study. Survival functions were estimated using the Kaplan-Meier method and compared by the log-rank test, and χ (2) tests were used for categorical data. Results: Among the 87 DLBCL patients treated with RCDOP regimen, 81 patients achieved complete remission (CR) or partial remission (PR), with ORR as 93.1%. Patients were further classified into groups, according to the risk factors, such as IPI scores, multiple extra-nodal involvement, bulky disease, age>60, tumor Ki-67>80%, elevated serum LDH level and advanced Ann Arbor stage. The progression-free survival (PFS, P =0.084) and overall survival (OS, P =0.515) had no statistical difference among the IPI low risk (0-1 score) group, intermediate risk (2-3 scores) group and high risk (4-5 scores) group. Similarly, no statistical difference were fou nd in PFS and OS of patients with extra-nodal involvements ≥2 ( P =0.303 and P =0.624), with bulky disease ( P =0.518 and P =0.466), with age>60 ( P =0.600 and P =0.183), with elevated serum LDH level ( P =0.054 and P =0.880), with advanced Ann Arbor stage ( P =0.075 and P =0.286), and with tumor Ki-67 over 80% ( P =0.190 and P =0.109), when compared with those of patients without these risk factors. Conclusion: RCDOP can improve the therapeutic effect and prognosis of DLBCL patients with certain high risk factors, such as intermediate and high IPI risks, multiple extra-nodal involvements, bulky disease, age over 60, elevated LDH level, advanced Ann Arbor stage and tumor Ki-67 over 80%.

Objective: To evaluate the efficacy and prognostic factors of second-line regimens for patients with relapsed/refractory diffuse large B-cell lymphoma (DLBCL). Methods: A retrospective analysis was performed in 98 patients with relapsed/refractory DLBCL who were treated with salvage regimens in Rui Jin Hospital from July 2004 to June 2016. Overall response rate (ORR) was evaluated after all treatment finished. Overall survival (OS) was analyzed by Kaplan-Meier method and multivariate by Cox proportional hazards models. Results: There were 60 males and 38 females with a median age of 55.5 (15-77) years. 48 (49.0%) patients responded to chemotherapy, and 32 (32.7%) patients achieved complete remission (CR). Factors affecting ORR were progression disease or refractory/relapse status less than 12 months after diagnosis ( χ (2)=5.878, P =0.015) , IPI intermediate-high/high risk ( χ (2)=5.930, P =0.015) and NCCN-IPI intermediate-high/high risk ( χ (2)=4.961, P =0.026). No significance difference was observed in ORR between germinal-center B-cell type (GCB) and non-GCB ( χ (2)=0.660, P =0.417). One-year and 2-year OS rates were 51.0% and 31.5%, with median OS at 13.17 months, respectively. Multivariate analysis indicated NCCN-IPI intermediate-high/high risk[ HR =2.176 (95% CI 1.338-3.538) , P =0.002] and response to chemotherapy [ HR =0.273 (95% CI 0.165-0.452) , P <0.001] were independent prognostic factors for survival. Conclusion: NCCN-IPI is a valid predictor of outcome for patients with relapse/refractory DLBCL. Response to chemotherapy is an independent prognostic factor for better survival.