A Rare Case of Helicobacter Pylori-Negative Gastric- MALT Lymphoma, Disseminated to the Small bowel, Colon, and Lung

Abstract

Extranodal marginal zone lymphomas of mucosa-associated lymphoid tissue (EMZL-MALT) have been linked to chronic immune stimulation due to infection or autoimmune stimuli. Helicobacter pylori induced gastric MALT lymphoma is the most extensively documented and well understood. However, in those where H. pylori is not detected, data is less established. Its treatment and prognosis are based on histological evaluation, immunophenotyping, and the use of fluorescence in situ hybridization (FISH), or polymerase chain reaction (PCR) to identify chromosomal translocation and/or unbalanced aberrations. The most common chromosomal translocation with pathogenic significance is t (11;18) (q21:q21). Those patients presenting with this translocation tend to have more advanced disseminated disease and are likely resistant to conventional antibiotic therapy and certain chemotherapeutic agents. Synchronous MALT lymphoma and overt distant dissemination has rarely been reported. We report a case of a 71-year-old female with biopsy confirmed H. pylori negative-gastric MALT lymphoma with disseminated disease involving the stomach, small intestine, colon, and lung. FISH analysis was positive for BIRC-3-MALT1 (11;18) fusion protein. Finally, we issue a call-to-action for much needed further data to establish clear treatment options based on clinical and diagnostic criteria, for extra-gastric, disseminated, and/or H. pylori negative MALT lymphomas. .