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The origin and targeting of mucosa-associated lymphoid tissue lymphomas

Martinez-Climent, Jose A.

Current Opinion in Hematology: July 2014 - Volume 21 - Issue 4 - p 309–319
doi: 10.1097/MOH.0000000000000051

Purpose of review Extranodal mucosa-associated lymphoid tissue (MALT lymphoma) is a distinct clinical–pathological entity that can be distinguished from other lymphomas by a number of unique features, including their location in various extranodal sites, being preceded by chronic inflammatory or infection processes; a characteristic histopathological picture; and the presence of exclusive chromosomal translocations which increase MALT1 proteolytic activity to promote constitutive NF-κB signaling and eventually drive lymphomagenesis.

Recent findings This review explores the major molecular and cellular events that participate in MALT lymphoma pathogenesis, focusing on gastric MALT lymphoma as a model of chronic inflammation-induced tumor development. In addition, the pivotal roles of activated MALT1 protease, its substrate TNFAIP3/A20, and the MyD88 adaptor protein in abnormally triggering downstream NF-κB pathway are overviewed. These new insights provide a mechanistic basis for using novel therapies targeting MALT1 protease or IRAK4 kinase activities. Finally, the putative cellular origin of MALT lymphomas is also discussed.

Summary Over the last decade, unraveling the biological complexity of MALT lymphomas has shed light on the fundamental cellular and molecular aspects of the disease that are to be translated into clinical diagnostics and therapy.

Division of Hematological Oncology, Center for Applied Medical Research, University of Navarra, Pamplona, Spain

Correspondence to Jose A. Martinez-Climent, MD, PhD, Division of Hematological Oncology, Center for Applied Medical Research (CIMA) Building, 1° Floor Lab 108, University of Navarra, Avda Pio XII, 55, Pamplona 31008, Spain. Tel: +34 948 194700x1029; fax: +34 948 194714; e-mail:

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