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Mucosal-Associated Lymphoid Tissue (MALT) Lymphoma

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Rare Lymphomas

Part of the book series: Hematologic Malignancies ((HEMATOLOGIC))

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Abstract

Mucosal-associated lymphoid tissue (MALT) lymphoma is a subtype of marginal zone lymphoma involving extranodal tissues. The most common site is the stomach, but MALT lymphoma has been described in a number of different organs and tissues including the skin, salivary glands, lung, small bowel, ocular adnexa, breasts, bladder, thyroid, dura, and synovium. First described in 1983 by Isaacson and Wright, it became a recognized entity approximately 10 years later by the International Lymphoma Study Group in the Revised European-American Lymphoma (REAL) classification in 1994 (Harris et al. Blood 84:1361–1392, 1994). Initially considered a “pseudo-lymphoma” because it appeared to remain dormant in one site for an extended period of time, MALT is now appreciated to be a clonal B-cell neoplasm and is disseminated at diagnosis in approximately one-third of all patients (Raderer et al. J Clin Oncol 24:3136–3141, 2006). It has been associated with a variety of chronic inflammatory and infectious conditions, including autoimmune diseases such as Sjögren’s syndrome and Hashimoto’s thyroiditis, and infections with Helicobacter pylori (H. pylori), Borrelia burgdorferi (B. burgdorferi), Chlamydophila psittaci (C. psittaci), Campylobacter jejuni (C. jejuni), and Hepatitis C virus (HCV) (Ambrosetti et al. Br J Haematol 126:43–49, 2004; Ferreri et al. J Natl Cancer Inst 96:586–594, 2004; Hussell et al. J Pathol 178:122–127, 1996). MALT lymphoma is an indolent B-cell non-Hodgkin lymphoma and principally observed until symptoms or organ impairment becomes evident; however, in some cases with early stage disease treatment with radiation therapy or antibiotic therapy may be curative. There are few dedicated studies of MALT lymphoma outside of early stage disease, and much of the management of advanced stage disease is extrapolated from the follicular lymphoma literature, which often includes a small number of marginal zone lymphoma patients. This chapter will review the epidemiology, biology and disease associations, clinical presentation and prognosis, and management of MALT lymphomas.

Pathology: Jan Delabie and Scott Rodig

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  1. Dana-Farber Cancer Institute, Boston, MA, USA

    Caron A. Jacobson MD & Ann S. LaCasce MD

  2. Division of Hematology Oncology, Department of Molecular Medicine, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy

    Luca Arcaini MD

  3. Department of Pathology, Oslo University Hospital, Oslo, Norway

    Jan Delabie MD

  4. Department of Pathology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA

    Scott Rodig MD

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  1. Medizinischen Klinik und Poliklinik III, Universität München Klinikum Großhadern, München, Germany

    Martin Dreyling

  2. Division of Hematology/Oncology and Cancer Center, University of Virginia Health System, Charlottesville, Virginia, USA

    Michael E. Williams

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Jacobson, C.A., Arcaini, L., LaCasce, A.S., Delabie, J., Rodig, S. (2014). Mucosal-Associated Lymphoid Tissue (MALT) Lymphoma. In: Dreyling, M., Williams, M. (eds) Rare Lymphomas. Hematologic Malignancies. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-39590-1_14

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