Marginal zone lymphoma (MZL) is a relatively uncommon subtype of non-Hodgkin's lymphoma, which can be categorized into three types: splenic MZL, nodal MZL (NMZL), and extranodal MZL (EMZL) of mucosa-associated lymphoid tissue (MALT). Extranodal MZL originates from extranodal organs and presents with diverse clinical manifestations depending on the affected organ. This retrospective study reviewed 104 patients who were diagnosed and treated for EMZL at the Department of Hematology in Peking University Third Hospital from 2014 to 2023, and had undergone at least one cycle treatment and evaluation. The median follow-up duration was 36 months. Among these patients, 48.1% were male, with a median age of 60 years. The stomach was the most frequently involved organ, followed by the lung and colon. The overall survival at 3-year and 5-year for the entire cohort were found to be 93.4% and 89.6%, respectively, while the progression-free survivals were determined as 78.2% and 58.3%. Survival analysis revealed that presence of B symptoms as well as progression of disease within 24 months (POD24) significantly influenced patient prognosis. Further investigation demonstrated the association between detectable monoclonal immunoglobulin protein (M protein) levels during disease onset in relation to tumor burden among patients. A total of 32 patients underwent next-generation sequencing analysis of tumor tissue; KEGG enrichment analysis showed that mutated genes primarily enriched pathways such as “PD-L1 expression and PD-1 checkpoint pathway in cancer,” “Jak-STAT signaling pathway”,and “Th1and Th2 cell differentiation.”

Disclosures

No relevant conflicts of interest to declare.

This content is only available as a PDF.
Sign in via your Institution