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MALT lymphoma (MONDO:0007650) is defined in the packet as an indolent, extranodal type of non-Hodgkin lymphoma composed of small B-lymphocytes (centrocyte-like cells), citing WHO 2001. The gastrointestinal tract is described as the most common site of involvement. Other common sites named in the definition are the lung, head and neck, ocular adnexae, skin, thyroid and breast. Gastric involvement is described as associated with the presence of H. pylori infection. The packet lists subtype entries for thyroid gland, breast, small intestinal and gastric mucosa-associated lymphoid tissue lymphoma, as well as tonsillar lymphoma. The prevalence record places the condition in the UNCOMMON category, with an estimate of 1-9 in 100,000; the packet does not state the population, geography or whether this is a birth or point prevalence. Affected organ systems are recorded as blood and immune, endocrine and respiratory.
The packet's phenotype annotations (18 recorded) carry HPO frequency categories. Features in the 80-99% (very frequent) category are fever, anemia, nausea and vomiting, pulmonary infiltrates, fatigue, B-cell lymphoma, hyperhidrosis and weight loss. Constipation is listed in the 30-79% (frequent) category. Features in the 5-29% (occasional) category are posterior uveitis, abnormality of the thyroid gland, mediastinal lymphadenopathy, abnormal nasolacrimal system morphology, abdominal pain, recurrent respiratory infections, lymphadenopathy and visual impairment. These annotations are population-level frequency labels and do not describe any individual's course. The packet does not supply age-of-onset categories, and it does not include a GeneReviews clinical description. The definition's list of involved sites (gastrointestinal tract, lung, head and neck, ocular adnexae, skin, thyroid and breast) is given, but the packet does not link specific features to specific sites.
The packet records one gene, BCL10 (chromosome 1), as associated with this condition. No ClinGen gene-disease validity classification is supplied for BCL10, so the strength of the association is not certified here. One synonym in the packet is 'lymphoma, MALT, somatic', but the packet gives no molecular mechanism, so how BCL10 relates to the condition is not described in this packet. The inheritance field lists MULTIFACTORIAL; the packet supplies no recurrence figures, penetrance or other inheritance details. The definition states that gastric involvement is associated with H. pylori infection; this is an association as stated by the source, and the packet does not describe a causal pathway. The packet does not supply other risk factors.
The packet contains no diagnostic methods, diagnostic criteria or GeneReviews diagnosis content for this condition, so no testing approach is certified here. The phenotype annotations are descriptive frequency data and are not diagnostic criteria. No newborn screening (RUSP) information is recorded. The absence of diagnostic information in this packet reflects what was supplied and does not indicate that no diagnostic approaches exist.
The approved-treatment record in the packet lists one product with an active FDA market status: lenalidomide (REVLIMID), approved through an NDA reviewed by CDER, with an approval date of 2005-12-27. The same product appears in the orphan-drug records with the indication 'treatment of extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue'. The packet does not state the full labelled indications of the approval. The orphan-drug records also contain entries labelled DESIGNATED, WITHDRAWN or APPROVED for several other agents, including some entries whose listed indications concern other lymphomas or a non-lymphoma condition. Orphan designation is not FDA approval, and only the lenalidomide record is certified in the approved-treatment field as a current FDA-approved option in this packet. The packet supplies no foundational therapies, no GeneReviews management content and no information on treatment selection, staging or care teams, so none is described here.
98 trials found
Prognosis is not certified in this packet. No natural history or survival information is supplied, and the definition's description of the condition as indolent is the only course-related statement available.
The packet's clinical-trial summary indicates that active ClinicalTrials.gov records exist for the search term MALT lymphoma, with ten detailed records supplied. Examples include NCT07029217, a Phase 3 study of reduced dose radiation therapy for people with B-cell lymphomas (Memorial Sloan Kettering Cancer Center; recruiting; start 2025-06-09, completion 2028-06-01), and NCT05100862, a Phase 3 study comparing zanubrutinib plus anti-CD20 with lenalidomide plus rituximab in relapsed or refractory follicular or marginal zone lymphoma (BeOne Medicines; active, not recruiting; start 2022-03-10, completion 2030-06-01). Other records include Phase 1 and Phase 2 studies and two records without a listed phase. Several listed studies address B-cell malignancies or lymphomas more broadly than this specific condition, and trial titles are not claims about the effect of any intervention. The packet's publication digest classifies 624 items, with case reports and case series as the dominant type, and notes the presence of gene therapy, biomarker and recent trial publications.
Data assembled from 8 of 12 sources · Last updated Nov 4, 2026, 9:30 AM UTC
Online Mendelian Inheritance in Man
European rare disease database
Genetic and Rare Diseases Info Center
Common questions about MALT lymphoma
AI-curated news mentioning MALT lymphoma
Updated Sep 12, 2026
A recent study highlights a rare case of MALT lymphoma presenting with simultaneous involvement of the lacrimal gland and stomach, as observed through (18)F-FDG PET/CT imaging. This case contributes to the understanding of MALT lymphoma's diverse presentations.
A case series reports five instances of primary pulmonary mucosa-associated lymphoid tissue (MALT) lymphoma exhibiting atypical imaging findings. This study contributes to the understanding of MALT lymphoma's presentation and may influence diagnostic approaches.
A recent study highlights primary pulmonary MALT lymphoma as a rare condition often misdiagnosed due to its pneumonic presentation. This research underscores the need for increased awareness and accurate diagnosis of this rare lymphoma subtype.