Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue (MALT Lymphoma)

Abstract

Extranodal marginal zone lymphoma of the mucosa-associated lymphoid tissue (MALT lymphoma) is a distinct lymphoma entity in the WHO-classification and accounts for up to 8% of all B-cell lymphomas. It displays unique clinical and pathological features and is associated with chronic infections including Helicobacter pylori (HP) and Chlamydophila psittaci (CP), as well as autoimmune diseases. MALT lymphoma is an indolent disease as reflected by a 10-year overall survival of 75–80%. In patients with systemic disease, a simple prognostic index (MALT-IPI) including age, stage and LDH has been shown to discriminate between three prognostic risk-groups. Antibiotic therapy is the treatment of choice in gastric MALT lymphoma as well as in some cases of ocular adnexal MALT lymphoma (OAML). HP eradication results in response rates of 80% in gastric MALT lymphoma and 33–65% in OAML. Additionally, radiation and systemic treatment, including anti-CD20 antibodies, as well as chemotherapy and chemo-free approaches are highly effective in the management of these patients.KeywordsMarginal zone lymphomaMALT lymphomaExtranodal lymphomasLymphomasNon-Hodgkin lymphoma Helicobacter pylori