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Background: Ocular adnexal marginal zone B-cell lymphoma (OAMZL) has been associated with Chlamydophila psittaci , an infection that may be transmitted by carrier animals. However, it is still unclear whether exposure to animals affects the risk of OAMZL in comparison with other lymphoma histotypes. We therefore investigated the role of professional and/or domestic exposures to animals in the occurrence of OAMZL, as compared with other types of lymphoma. Methods: A hospital-based case–control study was carried out on 43 consecutive OAMZL patients (cases) and 87 consecutive patients with nodal non-Hodgkin's lymphomas (NHLs; controls). Multiple logistic regression (MLR) odds ratios (ORs), and 95% confidence intervals (CIs) were used to estimate the association between exposures to animals and OAMZL risk. Results: A higher proportion of cases reported a lifetime exposure to household animals (79.1% vs 64.4% among controls), with a non-statistical significant MLR-OR of 2.18 (95% CI: 0.85–5.62). The OAMZL cases more frequently reported a history of occupation in breeding and/or slaughtering than controls (34.9% vs 6.9%), with an overall increased risk of 7.69 (95%CI: 2.65–22.34). Conclusion: These results indicate that, compared with nodal NHLs, the risk of OAMZL is markedly increased by contact with animals, particularly by occupational exposures.

The third most common lymphoma, MZL is usually indolent and often a consequence of chronic antigenic stimulation from a pathogen such as Helicobacter pylori or hepatitis C virus. Some MZLs regress with treatment of the infection. Patients with genetic lesions are treated with chemoimmunotherapy.

We aimed to investigate prognostic determinants of extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma). We analyzed 11,730 patients captured in the National cancer registry. Kaplan–Meier estimates compared survival by sex, age, and SEER stage, while multivariable Cox models identified mortality determinants. Women represented 54.6% of cases and had better survival than men. Age distribution peaked in the fifth–sixth decades. Survival declined with older age and advanced SEER stage. In multivariable models, male sex (hazard ratio [HR] 2.00, 95% confidence interval [CI] 1.48–2.70), advanced age (HR 33.92, 95% CI 18.23–63.10 for ≥ 80 years), and metastatic disease (HR 2.77, 95% CI 2.03–3.78), diabetes, cardiovascular, and cerebrovascular disease were associated with higher mortality. Conversely, higher household income (HR 0.63, 95% CI 0.45–0.88), physical activity (HR 0.60, 95% CI 0.42–0.86), and higher body mass index (HR 0.95, 95% CI 0.91–0.99) were associated with reduced mortality. Subgroup analyses showed consistently worse outcomes for men across age strata. Comorbidity effects were most pronounced at ages 60–79. Income, physical activity, smoking, and BMI have age-specific survival benefits. In conclusion, prognosis in MALT lymphoma is strongly influenced by sex, age, stage, comorbidities, and lifestyle.

Although the majority of lymphomas are diagnosed in lymph nodes, bone marrow, or other viscera, initial diagnosis of systemic lymphomas in the skin is a rare but important occurrence in dermatology. This study seeks to quantify the incidence of initial skin presentation in patients with systemic B-cell lymphomas (BCL) via examination of data in the Surveillance, Epidemiology, and End Results (SEER)-18 database; cases of primary cutaneous B-cell lymphoma were excluded. We found that an initial diagnosis of lymphoma in the skin is a very rare occurrence for systemic B-cell lymphomas, comprising < 0.3% of cases overall. Follicular lymphoma was the most likely to be diagnosed in the skin (1.47%), followed by marginal zone lymphoma (MZL, 0.5%), mantle cell lymphoma (0.4%), diffuse large B-cell lymphoma (DLBCL, 0.23%), Burkitt lymphoma (0.23%), Hodgkin lymphoma (0.04%), and chronic lymphocytic leukemia (0.006%). While indolent systemic lymphomas (MZL and FL) presenting initially in the skin have a better prognosis than those presenting at other sites, the more aggressive systemic DLBCL presenting in the skin does not demonstrate improved prognosis.

Primary gastric lymphoma (PGL) represents a rare pathology, which can be easily misdiagnosed because of unspecific symptoms of the digestive tract. Histologically, PGL can vary from indolent marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT) to aggressive diffuse large B-cell lymphoma (DLBCL). During the years, clinical trials revealed the important role of Helicobacter pylori (H. pylori) in the pathogenesis of gastric MALT lymphoma. Infection with Helicobacter pylori is an influential promoter of gastric lymphomagenesis initiation. Long-term studies revealed that eradication therapy could regress gastric lymphomas.

Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) is the third most prevalent subtype of mature B-cell lymphomas. While MALT lymphoma typically has a favorable prognosis, the outlook can worsen dramatically if histological transformation (HT) occurs, usually into a more aggressive form like diffuse large B-cell lymphoma (DLBCL). Large-scale study with long-term follow-up specifically focused on the transformation of MALT lymphoma is limited. This study utilized the SEER database to analyze 20,944 patients diagnosed with MALT lymphoma from 2000 to 2020. We also included a cohort of de novo DLBCL patients for comparison. Our findings showed that HT occurred in 1.59% of MALT lymphoma cases, with 5- and 10-year cumulative incidences of 1.2% and 2.2%, respectively. Patients presenting with B symptoms, age ≥ 60, and advanced-stage disease were at a significantly higher risk for HT. The median time to transformation was 3.67 years. Both univariate and multivariate analyses demonstrated that age ≥ 60 was a significant adverse factor for lymphoma-specific survival (LSS) and overall survival (OS) following HT. This population-based study contributes to the characterization of transformation risk and outcomes in MALT lymphoma and may help inform clinical surveillance strategies.

•Diffuse-large B-cell and marginal zone lymphoma: inflammatory conditions increase risk.•Myeloma, follicular lymphoma and chronic lymphocytic leukaemia: inflammatory conditions do not impact on risk.•Inflammatory conditions are far more common in women, but gender is not an effect modifier.•Inflammatory co-morbidities do not impact adversely on mature B-cell malignancy survival.•The increased risk for diffuse large B-cell lymphoma is not mediated by the activated B-cell subtype. Autoimmune inflammatory disease increases the risk of diffuse large B-cell lymphoma (DLBCL) and marginal zone lymphoma (MZL), but findings for other mature B-cell malignancies are equivocal. Furthermore, it has been suggested that the increase in DLBCL is due to the activated B-cell (ABC) subtype; but data on this, and the impact of inflammatory co-morbidities on survival, are sparse and contradictory. Data are from an established UK population-based cohort. Patients (n = 6834) diagnosed between 01/2009 and 08/2015 are included; DLBCL (n = 1771), myeloma (n = 1760), chronic lymphocytic leukaemia (CLL, n = 1580), MZL (n = 936), and follicular lymphoma (FL, n = 787). Information on rheumatological disorders and deaths was obtained by record-linkage to nationally compiled Hospital Episode Statistics, with age-and sex-matched individuals (n = 68,340) from the same catchment population (˜4 million people) providing the comparator. Significantly increased risks for DLBCL (OR = 2.3, 95% CI 1.8–2.8) and MZL (OR = 2.0, 95% CI 1.5–2.7) were found for those with rheumatological disorders; the site distribution of those with/without rheumatological conditions differing for DLBCL (p = 0.007) and MZL (p = 0.002). No increases in risk were observed for the remaining mature B-cell malignancies, and no associations with survival were detected for DLBCL (age-adjusted HR = 1.2, 95% CI 0.9–1.6) or MZL (age-adjusted HR = 1.0, 95% CI 0.6–1.9). Furthermore, whilst our findings provide evidence for an association with rheumatological disease severity for DLBCL, they offer little support for the notion that the association is driven by an increase in the incidence of the ABC subtype. Our findings support the hypothesis that the chronic activation and proliferation of specific B-cell populations which characterize autoimmune disease increase the potential for the lymphomagenic events that lead to DLBCL and MZL in both males and females; but have no impact on the development of CLL, FL or MM, or on survival.

BackgroundOphthalmic lymphoma (OL) is the most common orbital tumour, particularly in older individuals. Little is known about the epidemiology and geographic distribution of OL in Canada. Descriptive demographic statistics are an important first step in understanding OL burden and are necessary to inform comprehensive national cancer prevention programmes.MethodsWe determined patterns of incidence and geographical distribution of the three major subtypes of OL: extranodal marginal zone B cell lymphoma, follicular lymphoma (FL) and diffuse large B cell lymphoma. Here, we used cases that were diagnosed during 1992–2010 using two independent population-based cancer registries, the Canadian Cancer Registry and Le Registre Québécois du Cancer (LRQC).ResultsThe OL mean annual age-standardised incidence rate for 1992–2010 was 0.65 cases per million people per year with an average annual increase in the incidence rate of 4.5% per year. The mean age of diagnosis was 65 years. OL incidence rate was the highest in the cities located along the heavily industrialised Strait of Georgia in British Columbia.ConclusionsOur data on patient age, sex and temporal trends showed similarities with data reported in the USA and Denmark. Additional studies are needed to determine whether the observed increase in OL incidence is genuine or spurious.

Primary head and neck mucosa-associated lymphoid tissue lymphoma (HN-MALT) is a rare lymphoma with unknown incidence and prognosis. We allocated HN-MALT data from the Self-Surveillance, Epidemiology, and End Results database (2000–2021) into training and validation cohorts at a 7:3 ratio. A joinpoint regression analysis was used to examine sex-specific and age-group morbidities, and independent prognostic factors were identified through multivariate Cox analysis to construct a nomogram prediction model and verify the accuracy of prediction. A total of 2,517 patients with HN-MALT were randomly divided into training (1,762) and validation (755) groups. The incidence of HN-MALT in men and women decreased from 2007 to 2021, with an annual percent change (APC) of -3.04% (95%: -4.4 − -1.6, P  < 0.05), while in the ≥ 60 years old group, the incidence decreased significantly from 2016 to 2021, with an APC of -8.20 (95%: -13.9 − -2.1, P  < 0.05). The multivariate Cox analysis revealed that male, ≥ 60 years old, white, and divorced/separated/widowed were independent risk factors of the overall survival and lymphoma-specific survival. A nomogram prediction model was constructed based on the Cox regression results for multiple factors, the areas under the curve, the calibration curve, and decision curve analysis results of which indicated that the nomogram prediction model performed very well. HN-MALT cells exhibit distinctive clinical and pathological characteristics. Furthermore, we developed a nomogram model for the prognostic assessment to offer valuable guidance for clinical decision-making.

Background/AimsLymphomas are the most frequent neoplasm of the orbit. However, the epidemiology of orbital lymphomas is not well reported. This study aimed to provide a population-based report on the epidemiology of orbital lymphomas and measure the trends in the incidence of orbital lymphoma cancer in South Korea.MethodsNationwide cancer incidence data from 1999 to 2016 were obtained from the Korea Central Cancer Registry. Age-standardised incidence rates and annual percent changes were calculated according to sex and histological types. The analysis according to the Surveillance, Epidemiology, and End Results summary stage classifications was performed from 2006 to 2016. Survival rates were estimated for cases diagnosed from 1999 to 2016.ResultsA total of 630 patients (median age: 54 years) with orbital lymphoma in the orbital soft tissue were included in this study. The age-standardised incidence rates increased from 0.03 to 0.08 per 100 000 individuals between 1999 and 2016, with an annual percent change of 6.61%. The most common histopathological type of orbital lymphoma was extra marginal zone B cell lymphoma, accounting for 82.2% of all orbital lymphomas during 1999–2016, followed by diffuse large B cell lymphoma (9.2%). Five-year, 10-year and 15-year overall survival (OS) of orbital lymphoma was 90.8%, 83.8% and 75.8%, respectively. OS showed a significant decrease as age increased and no significant differences between men and women.ConclusionThe incidence rate of orbital lymphoma is very low in South Korea. However, the incidence rate has increased over the past years. Orbital lymphomas have a worse prognosis as age increases.