HaematologyTier 2Disease (DEADMAN)

MALT lymphoma

Description

MALT lymphoma (extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue) - an indolent B cell non-Hodgkin lymphoma arising at extranodal mucosal sites, most classically the stomach, where it is strongly associated with chronic *Helicobacter pylori* infection

The most common extranodal marginal zone lymphoma; gastric MALT lymphoma is the classic and most treatment-informative presentation, though MALT lymphoma can arise at other mucosal sites (salivary gland, thyroid, ocular adnexa, lung)

Chronic antigenic stimulation (from H. pylori in the stomach, or other chronic inflammatory/autoimmune triggers at other sites - e.g. Sjogren's syndrome for salivary gland MALT lymphoma, Hashimoto's thyroiditis for thyroid MALT lymphoma) drives sustained B cell proliferation within mucosal lymphoid tissue, which can progressively acquire additional genetic changes and become a clonal lymphoma - early-stage disease can remain genuinely "antigen-driven" and reversible with elimination of the trigger

Gastric MALT lymphoma diagnosed via endoscopic biopsy; *staging includes formal H. pylori testing* (biopsy urease test, histology, or non-invasive testing) given its direct treatment relevance
t(11;18) translocation is associated with H. pylori-independent disease that is *less likely to respond to H. pylori eradication alone* - a specific, clinically actionable cytogenetic finding

*Early-stage, H. pylori-positive gastric MALT lymphoma: H. pylori eradication therapy alone* produces complete remission in the majority of cases - a distinctive feature not seen in most other lymphomas, where antibiotic treatment is curative rather than chemotherapy
Disease not responding to eradication (including t(11;18)-positive or H. pylori-negative disease), or more advanced-stage disease, is managed with radiotherapy (for localised disease) or systemic therapy (rituximab +/- chemotherapy) as for other indolent lymphomas

Helicobacter pylori infection, Sjogren's syndrome (salivary gland MALT lymphoma), Hashimoto's thyroiditis (thyroid MALT lymphoma), gastric adenocarcinoma (a separate H. pylori-associated malignancy)

Gastric MALT lymphoma is one of the clearest examples in oncology of a malignancy that can be cured by treating an infection rather than with cytotoxic therapy - a direct consequence of its antigen-driven pathogenesis at the early, still-reversible stage

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