IBCL-618: Perspective on marginal zone lymphoma in India

Abstract

Context: Marginal zone lymphomas (MZL) are indolent non-Hodgkin lymphomas (NHL), representing 7% to 17% of NHL cases in Western nations. MZL consists of three subtypes: extranodal mucosa-associated lymphoid tissue (MALT lymphoma), nodal MZL, and splenic MZL. Objective: Data on the incidence and prognosis of MZL in patients from India are nonexistent. Design, Setting, and Participants This retrospective study aims to examine patients who were diagnosed with NHL and were registered at the Department of Medical Oncology, AIIMS, New Delhi. There were 1,800 confirmed cases of NHL in adults (individuals ≥18 years) between 2014 and 2023. Data on baseline features, therapy, and survival were extracted from patient medical records. Active surveillance, antibiotic monotherapy, radiotherapy, single-agent rituximab, or rituximab plus chemotherapy were the recommended treatments. Results: Out of all NHL cases, 54 (3%) were classified as MZL. The cohort comprised 33 males and 21 females, with a median age of 59 years (range: 21–89 years). MALT accounted for 30 cases (56%), splenic MZL for 14 cases (26%), and NMZL for 10 cases (19%)—representing the three most prevalent subtypes. Moreover, 6 individuals (11%) exhibited elevated LDH levels. The gastric region represented 60% of MALT lymphoma cases, followed by the ophthalmic area at 20%, and the parotid gland at 10%; of the remaining cases, 4 were associated with the intestinal region and 1 each for breast, lung, skin, and brain. In the follow-up, 42.5% of patients received chemo-immunotherapy, comprising bendamustine and rituximab; 7.4% were treated with single-agent rituximab; 21% underwent radiation therapy; and nearly 33% remained under close observation. Approximately 63% of the cases underwent combined modality treatment, which included antibiotics, radiation, chemoimmunotherapy, or SA rituximab. The response rate following treatment was 83%. Within the entire cohort, 7.4% exhibited transformed high-grade lymphoma. At a median follow-up of 40 months, the event-free survival was 72.1%, while the overall survival rate was 85.4%. Conclusion: MZL represents 3% of the total NHL. Extranodal marginal zone lymphoma (ENMZL) was the most prevalent, followed by splenic MZL. The response rate and outcome were consistent with existing literature.