The Korean Journal of Medicine

The Korean Association of Internal Medicine (대한내과학회)
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Pathophysiology of Gastric MALT Lymphoma

위 MALT 림프종의 병태생리

  • Park, Gyeongsin (Department of Hospital Pathology, The Catholic University of Korea College of Medicine) ;
  • Kang, Chang Suk (Department of Hospital Pathology, The Catholic University of Korea College of Medicine)
  • 박경신 (가톨릭대학교 의과대학 병원병리학교실) ;
  • 강창석 (가톨릭대학교 의과대학 병원병리학교실)
  • Published : 2012.12.01
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Abstract

Early gastric mucosa-associated lymphoid tissue (MALT) lymphoma is considered as an antigen-dependent disease associated with long standing antigenic stimulation by Helicobacter pylori (H. pylori) which induces chronic immune response and lymphoid tissue development at the gastric mucosa normally devoid of lymphoid tissue. With disease progression, antigen-independent clones occur via genetic alterations inducing aberrant activation of nuclear factor ${\kappa}B$ $(NF-{\kappa}B)$ pathway which is essential for regulation of normal lymphocyte development and activation. Four major translocations, including t (11;18)/API2-MALT1, t (1;14)/BCL10-IGH, t (14;18)/(IGH-MALT1 and t (3;14)/FOXP1-IGH, occur mutually exclusively and lead to generation of cIAP2-MALT1 fusion protein or overexpression of BCL10, MALT1 and Foxp1. Translocation t (3;14)(q27;q32)/BCL6-IGH and t (1;2)(p22;p12)/$BCL10-IG{\kappa}L$ also occur in some MALT lymphomas. Mutational inactivation of A20, global $NF-{\kappa}B$ inhibitor, involve the development of especially translocation-negative MALT lymphoma. Downstream effects of most genetic alteration converge on the same $NF-{\kappa}B$ mediated oncogenic pathway. This review discusses the current advances in the pathophysiology underlying the development of gastric MALT lymphoma and its progression.

Keywords

References

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