Abstract
Simple SummaryExtranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), which is frequently linked to chronic antigenic stimulation, is clinically an indolent B-cell lymphoma. The most common site is the stomach, accounting for 35% of MALT lymphomas, with a strong association with Helicobacter pylori (H. pylori) infection. Although antibiotic eradication is first-line therapy for gastric MALT lymphoma, a recent trend showing increasing H. pylori-negative cases may require different treatment strategies. Intestinal MALT lymphoma, including immunoproliferative small intestinal disease, is relatively rare, and the pathogenesis and therapeutic approach have not been fully elucidated. In addition, the gastrointestinal tract was recently considered a preferable site of Epstein–Barr-virus-negative marginal zone lymphoma in the post-transplant setting. We review the updated clinicopathological features, treatment, and evolving concepts of MALT lymphoma of the gastrointestinal tract.Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) typically arises from sites such as the stomach, where there is no organized lymphoid tissue. Close associations between Helicobacter pylori and gastric MALT lymphoma or Campylobacter jejuni and immunoproliferative small intestinal disease (IPSID) have been established. A subset of tumors is associated with chromosomal rearrangement and/or genetic alterations. This disease often presents as localized disease, requiring diverse treatment approaches, from antibiotic therapy to radiotherapy and immunochemotherapy. Eradication therapy for H. pylori effectively cures gastric MALT lymphoma in most patients. However, treatment strategies for H. pylori-negative gastric MALT lymphoma are still challenging. In addition, the effectiveness of antibiotic therapy has been controversial in intestinal MALT lymphoma, except for IPSID. Endoscopic treatment has been noted to usually achieve complete remission in endoscopically resectable colorectal MALT lymphoma with localized disease. MALT lymphoma has been excluded from post-transplant lymphoproliferative disorders with the exception of Epstein–Barr virus (EBV)-positive marginal zone lymphoma (MZL). We also describe the expanding spectrum of EBV-negative MZL and a close association of the disease with the gastrointestinal tract.
Highlights
Marginal zone B-cell lymphoma (MZL) is a group of indolent B-cell lymphomas originating from memory B cells normally present in a distinct compartment, known as the marginal zone of secondary lymphoid tissues
The presence of lymphoepithelial lesions (LELs) is suggestive of MALT lymphoma in the stomach, but this is not essential for this diagnosis because they can be seen in other low-grade B-cell lymphomas
The endoscopic findings on narrow band imaging (NBI) magnifying endoscopy were characterized by a tree-like appearance (TLA), which corresponded to abnormally large vessels resembling a tree trunk with long, bare branches [64]
Summary
Marginal zone B-cell lymphoma (MZL) is a group of indolent B-cell lymphomas originating from memory B cells normally present in a distinct compartment, known as the marginal zone of secondary lymphoid tissues. Other bacterial infections have been found to be implicated in the pathogenesis of MALT lymphoma arising in the skin (Borrelia burgdorferi), the ocular adnexa (Chlamydophilia psittaci), lungs (Achromobacter xylosoxidans), and the small intestine (Campylobacter jejuni). This association is variable in different geographical areas [3,4,5]. A link between C. jejuni infection and immunoproliferative small intestinal disease (IPSID) has been established, which is classified as a variant of MALT lymphoma, with marked plasma cell differentiation and a favorable response to antibiotic therapy [11,12]. We focus on the recent advances in the diagnosis and treatment of MALT lymphoma affecting the GI tract
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