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Objectives: The advanced extra‐nodal NK/T‐cell lymphoma (ENKTL) is highly aggressive and lacks effective treatment with a poor prognosis. This study aimed to investigate the effectiveness and safety of autologous hematopoietic stem cell transplantation (ASCT) in CR1. Methods: Forty of 121 patients with advanced ENKTL from four Chinese hospitals between January 2006 to December 2021 who achieved first complete remission (CR1) and received at least 4 cycles chemotherapy, were enrolled for analysis. Twenty patients received ASCT as up‐front consolidation therapy (Group A), and 20 patients only received chemotherapy (Group B). Clinical features, treatment and follow‐up information were collected. Results: With a median follow‐up of 27 months (range, 4–188 months), the 2‐year overall survival (OS) in Group A, 61% (95% CI 37%–85%), was better than that in Group B, 26% (95% CI 2%–50%), p =.018. The 2‐year progression‐free survival (PFS) was 56% (95% CI 32%–80%) in Group A, 26% (95% CI 2%–50%) in Group B, p =.026. III–IV grade hematological toxicity was the most common adverse event. No treatment‐related deaths were observed in both groups. Conclusion: Up‐front ASCT could improve survival of advanced ENKTL patients in first complete remission, but need be confirmed by a prospective clinical trial. [ABSTRACT FROM AUTHOR]

Background: To our knowledge, the different situations of identifying second primary malignant tumors (SPMTs) in lymphoma patients with synchronous solid tumors remain to be comprehensively investigated. Methods: We retrospectively collected information pertaining to lymphoma patients with synchronous solid tumors (diagnosed within 6 months) at Peking University Cancer Hospital & Institute between 2009 and 2019. The non‐parametric Aalen–Johansen estimator was applied to calculate cumulative incidence function in the competing risk model. Furthermore, propensity score‐matched analysis was performed to compare survival differences in lymphoma patients with or without synchronous solid tumors. Results: Thirty‐eight patients were enrolled. There were three situations of identifying SPMTs. First, in 15 patients (39.5%), SPMTs were identified before the initiation of any treatment. Among them, priority was given to anti‐lymphoma treatment in case of only three patients. Second, in 17 patients (44.7%), SPMTs were unexpectedly detected on surgical specimen assessment; of them, 13 received anti‐lymphoma treatment after surgery. Third, in six patients (15.8%), SPMTs were identified after the outset of treatment for the primary tumor; in this population, three of four patients with lymphoma switched toward the treatment plan for SPMTs. The 5‐year overall survival was 58.7%. The cumulative incidence function within 5 years was 26.6% for lymphoma and 14.7% for other solid tumors. The early identification of SPMTs was associated with better outcomes (p = 0.048). After balancing the baseline characteristics, no differences in survival were observed between lymphoma patients with and without synchronous solid tumors (p = 0.664). Conclusions: This is the first study to present the different situations of identifying SPMTs in lymphoma patients with synchronous solid tumors. In only <50% patients, SPMTs were identifiable at baseline. SPMT identification at different situations may make it difficult to choose the optimal therapeutic option, which may consequently impact patient survival. [ABSTRACT FROM AUTHOR]

: A primary breast lymphomais a rare form of extranodal lymphoma type. We aimed to analyze prognosticriskfactors and explore relapse factors in primary breast diffuse large B cell lymphoma (PB-DLBCL). : From November 2003 to September 2020, sixty-three patients from two medical centers newly diagnosed with PB-DLBCL patients were analyzed retrospectively. : The median age was 52, and >50% of patients were post-menopausal. The international prognostics index (IPI) (0–1) was mainlyin the low-risk group (84%), and there were four patients with stage IV (6%) who had bilateral breast involvement. With a median follow-up time of 4.92 years (3.17–8.00), five-year overall survival (OS) and progression-free survival (PFS) were 78.9% and 67.1%, respectively. Univariate and multivariate analyses showed that elevated erythrocyte sedimentation rate (ESR) and B symptoms were independent adverse prognostic risk factors for OS, whereas bilateral breast involvement was unfavorable for PFS. Disease recurrence and relapse occurred in 40% (25/63) patients, mainly in the breast, followed by the central nervous system (CNS) and skin/soft tissue. : This is the first study to explore the prognostic risk factors and relapse factorsof PB-DLBCL in a relatively large Chinese PBL cohort. Local breast and CNS recurrence after standard R-CHOP treatment were the main issues we are facing now. [ABSTRACT FROM AUTHOR]

Background: The optimal first-line treatment for extra-nodal NK/T-cell lymphoma (ENKTL) has not been well-defined. This study aimed to evaluate the efficacy and safety of pegaspargase, cyclophosphamide, vincristine, etoposide and prednisone (COEPL) regimen combined with radiotherapy for patients with newly diagnosed ENKTL. Methods: Our study is a prospective, open-label clinical trial. Patients with newly diagnosed ENKTL and an ECOG performance status of 0 to 2 were eligible for enrollment. For patients with stage I/II nasal ENKTL, treatment included 2 cycles of induction COEPL regimen followed by concurrent chemoradiotherapy, then by 2 cycles of COEPL regimen as consolidation. For patients with stage III/IV or primary extra-nasal ENKTL, treatment included 6-8 cycles of COEPL regimen with or without radiotherapy to local sites, and autologous stem cell transplantation was given in selected patients. Results: A total of 80 patients were enrolled. The median age was 41 years (range, 15-76 years). Sixteen patients (20%) had stage III/IV disease, and 10 (12.5%) had a PINK score≥2. Complete response and overall response rates were 75.9% and 87.3%, respectively. With a median follow-up of 41.4 months (range 2.7-76.2 months), the 3-year progression-free survival (PFS) and overall survival (OS) rates were 71.3% (95%CI 61.1-81.5%) and 73.3% (95%CI 63.1-83.5%), respectively. For patients with stage I/II nasal ENKTL (n=62), the 3-year PFS and OS were 78.1% and 81.2%, respectively. For patients with stage III/IV or primary extra-nasal ENKTL (n=18), 3-year PFS and OS were 48.1% and 45.7%, respectively. Major grade 3-4 adverse events were anemia (21.3%), leucopenia (22.5%), neutropenia (18.8%), and thrombocytopenia (7.6%). No treatment-related death was observed. Conclusions: Pegaspargase-COEP chemotherapy in combination with radiotherapy is highly effective and safe for patients with newly diagnosed ENKTL. [ABSTRACT FROM AUTHOR]

Extranodal natural killer (NK)/T cell lymphoma-associated hemophagocytic syndrome (HPS) (NK/T-LAHS) is a heterogeneous and life-threatening disease, which warrants investigation of its risk factors and clinical features. We retrospectively analyzed the clinical records of 202 patients with extranodal NK/T cell lymphoma and compared the characteristics and survival of extranodal NK/T cell lymphoma patients with and without HPS. The cumulative incidence of NK/T-LAHS was 11.4 % (23/202). In a multivariate logistic regression model, younger age (p = 0.012), bone marrow involvement (p = 0.012), and reduced serum albumin (p < 0.001) were independent risk factors for developing HPS in patients with extranodal NK/T cell lymphoma. The survival of extranodal NK/T cell lymphoma patients was aggravated when complicated with HPS, with an overall 2-year survival of 72.1 and 30.4 %, respectively (p < 0.001). Six patients with HPS onset at lymphoma diagnosis tended to have a poor performance status (p = 0.040), while the rate of elevated bilirubin was significantly higher in 17 patients with HPS onset at lymphoma relapse (p = 0.045). After HPS onset, treatment response was poor (response rate, 17.4 %) and survival was dismal with a median of 26 days. Univariate analysis showed that patients with lactate dehydrogenase >1000 U/L (p = 0.048) and disseminated intravascular coagulation (p = 0.004) had shorter survival time. Extranodal NK/T cell lymphoma was frequently complicated with HPS, and survival was discouraging in this circumstance. Intensive chemotherapy regimens including L-asparaginase or pegaspargase and allogeneic stem cell transplantation should be investigated. [ABSTRACT FROM AUTHOR]

There is no standard salvage regimen for patients with refractory and relapsed extranodal NK/T-cell lymphoma (NKTCL), nasal type. This study was conduced to evaluate the efficacy of L-asparaginase-based regimen as a salvage regimen, on refractory and relapsed extranodal NKTCL, nasal type. Between March 1996 and March 2008, 45 patients with refractory and relapsed extranodal NKTCL, nasal type, were studied retrospectively. All patients were treated with L-asparaginase-based salvage regimen. Thirty-nine patients also received primary involved-field radiation after L-asparaginase-based chemotherapy. The complete response rate, partial response rate, and overall response rate for the whole group were 55.6%, 26.7%, and 82.2%, respectively. Both of 3-year and 5-year overall survival (OS) rates were 66.9%. The major adverse effects of L-asparaginase were myelosuppression, liver dysfunction, hyperglycemia, and allergic reaction. In general, the side effects could be tolerated. On univariate analysis, age, the stage of disease, and performance status were found to be prognostic factors influencing OS. On multivariate analysis, the stage of disease and age were independent prognostic factors for OS. L-Asparaginase-based regimen was obviously effective for the patients with refractory and relapsed extranodal NKTCL, nasal type. [ABSTRACT FROM AUTHOR]

Background: The accurate information about lymphoma burden at national and provincial levels remains unknown in China. Methods: Following the general analytical strategy used in GBD 2016, the age-, sex-, and province-specific incidence, mortality, and prevalence of lymphoma in China were analyzed. Trends in the incidence, mortality, prevalence, and disability-adjusted life years (DALYs) due to Hodgkin's lymphoma (HL) and non-Hodgkin's lymphoma (NHL) were assessed from 2006 to 2016. Results: It was estimated that there were 75,400 new cases and 40,500 deaths of lymphoma in 2016 in China, of which 6900 new cases and 2900 deaths were due to HL, while 68,500 new cases and 37,600 deaths were due to NHL. The age-standardized incidence rate (ASIR), mortality rate (ASMR), and prevalence rate (ASPR) per 100,000 were 0.46, 0.19, and 1.75 for HL, and 4.29, 2.45, and 14.9 for NHL, respectively. An upward trend with age in incidence and mortality was observed. Males had higher incidence and mortality rates than females in all age groups. Sociodemographic index had a correlation with the ASIR (r = 0.75), ASMR (r = − 0.74), ASPR (r = 0.84), and age-standardized DALYs (r = − 0.75) of HL, as well as with the ASIR (r = 0.80), ASPR (r = 0.83), and age-standardized DALYs (r = − 0.33) of NHL. From 2006 to 2016, the age-standardized DALYs of HL decreased significantly, while the age-standardized DALYs of NHL increased from 2006 to 2013 and remained stable from 2013 to 2016. Conclusions: The burden of lymphoma in China showed unexpected patterns varied by sex, age, and provinces, with an increased trend of NHL and a decreased trend of HL from 2006 to 2016. [ABSTRACT FROM AUTHOR]