Synchronous Upper and Lower Gastrointestinal Mucosa-Associated Lymphoid Tissue Lymphomas

Abstract

Mucosa-associated lymphoid tissue lymphoma (MALToma) is a subtype of B-cell non-Hodgkin’s lymphoma, comprising ∼17% of all gastrointestinal (GI) tract lymphomas. It is associated with chronic inflammation and autoimmunity, for example Helicobacter pylori gastritis and Sjogren’s syndrome, respectively. Approximately 50% of GI MALTomas occur in the stomach, with small bowel and colonic lesions being less frequent. Synchronous upper and lower GI MALTomas occur rarely, with few cases reported. We present the case of a 73-year-old patient who presented with change in bowel habit and was found to have synchronous multifocal upper and lower GI MALTomas, which did not respond to H. pylori cure or to rituximab therapy, but did respond to a combination of surgery and chemotherapy with rituximab and bendamustine.

Helicobacter pylori and its association with autoimmune diseases: systemic lupus erythematosus, rheumatoid arthritis and Sjögren syndrome

“…Studies have linked H. pylori and SS with the development of multiple diseases, such as MALT lymphoma [ [91] , [92] , [93] , [94] , [95] ] and autoimmune pancreatitis [ 96 , 97 ], which could be incidental clinical findings, although they may share pathogenic molecular pathways such as the expression of CXCL13 and its CXCR5 receptor [ 98 , 99 ]. For example, in a study conducted in 9 SS patients with gastric MALT lymphoma, MALT lymphoma translocation 1 gene (MALT1) rearrangement was present in 78% of cases, and MALT1 is associated with resistance to H. pylori eradication therapy [ 100 ].…”

Multiple Synchronous Mucosa-Associated Lymphoid Lymphomas Involving in the Stomach, Duodenum, Ileum, and Sigmoid

“…Non-GI sites of MALToma include the lungs, salivary glands, thyroid gland, skin, and eyes. The presence of synchronous upper and lower GI MALTomas appears to be an extremely rare occurrence, with only a few cases reported [ 1 , 2 , 3 ]. This is the first report involving four separate synchronous MALTomas in the upper and lower GI tract.…”