MALT lymphoma pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

MALT lymphoma (MALToma) is a form of lymphoma involving the mucosa-associated lymphoid tissue (MALT), frequently of the stomach, but virtually any mucosal site can be afflicted. It is a cancer originating from B cells in the marginal zone of the MALT

Pathogenesis

MALT lymphoma (MALToma) is a form of lymphoma involving the mucosa-associated lymphoid tissue (MALT), frequently of the stomach, but virtually any mucosal site can be afflicted. It is a cancer originating from B cells in the marginal zone of the MALT

Genetics

  • Development of [MALT lymphoma is the result of multiple genetic mutations.
  • Genes involved in the pathogenesis of MALT lymphoma are tabulated below:[1]

Associated Conditions

  • Gastric MALT lymphoma is frequently associated with chronic inflammation as a result of the presence of Helicobacter pylori (72-98%).[2]
  • Chronic immune stimulation is also suspected in the pathogenesis of non-gastric MALT lymphoma, and hence often have a history of autoimmune disorders such as:

Gross Pathology

MALT lymphoma starts in the tissues or organs outside of the lymph nodes (extranodal). MALT lymphoma develops in mucosa-associated lymphoid tissue, in the mucosa, or tissue that lines body organs or body cavities including:[3]


MALT lymphoma pathophysiology
Endoscopic image of gastric MALT lymphoma taken in body of stomach in patient who presented with upper GI hemorrhage. Appearance is similar to gastric ulcer with adherent clot.

Microscopic pathology

Histologically, there is expansion of the marginal zone compartment with development of sheets of neoplastic small lymphoid cells.[4] The morphology of the neoplastic cells is variable with small mature lymphocytes, cells resembling centrocytes (centrocyte like cells), or marginal zone/monocytoid B cells. Plasmacytoid or plasmacytic differentiation is frequent. Lymphoid follicles are ubiquitous to MALT lymphoma but may be indistinct as they are often overrun or colonized by the neoplastic cells. Large transformed B cells are present scattered among the small cell population. If these large cells are present in clusters or sheets, a diagnosis of associated large B-cell lymphoma should be considered. A characteristic feature of MALT lymphoma is the presence of neoplastic cells within epithelial structures with associated destruction of the glandular architecture to form lymphoepithelial lesions.[5]

References

  1. Troppan, Katharina; Wenzl, Kerstin; Neumeister, Peter; Deutsch, Alexander (2015). "Molecular Pathogenesis of MALT Lymphoma". Gastroenterology Research and Practice. 2015: 1–10. doi:10.1155/2015/102656. ISSN 1687-6121.
  2. Parsonnet J, Hansen S, Rodriguez L, Gelb A, Warnke R, Jellum E, Orentreich N, Vogelman J, Friedman G (1994). "Helicobacter pylori infection and gastric lymphoma". N Engl J Med. 330 (18): 1267–71. PMID 8145781.
  3. Extranodal marginal zone of mucosa-associated lymphoid tissue (MALT lymphoma). Canadian Cancer Society 2016. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/malt-lymphoma/?region=on. Accessed on January 28, 2016
  4. Taal, B G; Boot, H; van Heerde, P; de Jong, D; Hart, A A; Burgers, J M (1 October 1996). "Primary non-Hodgkin lymphoma of the stomach: endoscopic pattern and prognosis in low versus high grade malignancy in relation to the MALT concept". Gut. 39 (4): 556–561. doi:10.1136/gut.39.4.556.
  5. Janusz, edited by Jankowski, (2012). Handbook of Gastrointestinal Cancer (2 ed.). Chicester: John Wiley and Sons Ltd. pp. 243–244. ISBN 978-0-470-65624-2.


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