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Lymphoma pp 111–126Cite as

MALT Lymphoma (Extranodal Marginal Zone B-Cell Lymphoma)

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Part of the book series: Current Clinical Oncology ((CCO,volume 43))

Abstract

Extranodal marginal zone lymphoma (MALT lymphoma) comprises approximately 8 % of non-Hodgkin’s lymphomas. Recurrent karyotype abnormalities, despite involving different genes, appear to affect the same signalling pathway, resulting in the activation of nuclear factor-kappa B (NF-κB). The most common site of MALT lymphoma is the stomach, although involvement may occur at any other site. MALT lymphomas mostly arise at sites normally devoid of lymphoid tissue and are often preceded by chronic inflammatory conditions. There is a convincing evidence of the pathogenetic role of Helicobacter pylori in gastric lymphoma, and other infectious agents may also have a pathogenetic role in other anatomical sites. H. pylori eradication with antibiotics can lead to the regression of localized gastric MALT lymphoma in over 75 % of patients. Treatment of non-gastric localizations with antibiotics remains mainly investigational. Patients who do not respond to antibiotic therapy may be considered for localized radiotherapy. Chemotherapy and immunotherapy can be effective in patients with disseminated disease. Several active drugs have been tested in phase II trials. The efficacy of the combination of rituximab with chlorambucil in either non-gastric or gastric antibiotic-resistant MALT lymphoma has been shown in a randomized study. Aggressive anthracycline-containing regimens are not usually necessary and should be reserved for the few patients with high tumor burden and for those with diffuse large-cell infiltration. These latter, indeed, should be treated according to the recommendations for diffuse large-cell lymphoma.

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Acknowledgments

This work was partially supported by the Fondazione per la Ricerca e la Cura sui Linfomi nel Ticino (Bellinzona, Switzerland) and Nelia et Amadeo Barletta Foundation (Lausanne, Switzerland).

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Authors and Affiliations

  1. Division of Research, Oncology Institute of Southern Switzerland (IOSI), Ospedale San Giovanni, Bellinzona, Ticino, 6500, Switzerland

    Emanuele Zucca MD

  2. Division of Research and Lymphoma and Genomics Research Program, Institute of Oncology Research (IOR), Via Vela 6, Bellinzona, Ticino, 6500, Switzerland

    Francesco Bertoni MD

  3. Division of Research, Oncology Institute of Southern Switzerland (IOSI), Ospedale San Giovanni, Bellinzona, Ticino, 6500, Switzerland

    Franco Cavalli MD, FRCP

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  1. Emanuele Zucca MD
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  2. Francesco Bertoni MD
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  3. Franco Cavalli MD, FRCP
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Correspondence to Emanuele Zucca MD .

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    Anas Younes

  2. Service d'Hematologie, CHU Lyon-Sud, chemin du Grand Revoyet, Pierre Bénite, 69310, France

    Bertrand Coiffier

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Zucca, E., Bertoni, F., Cavalli, F. (2013). MALT Lymphoma (Extranodal Marginal Zone B-Cell Lymphoma). In: Younes, A., Coiffier, B. (eds) Lymphoma. Current Clinical Oncology, vol 43. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-62703-408-1_6

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