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Review
. 2018 Mar 28:7:406.
doi: 10.12688/f1000research.13826.1. eCollection 2018.

Recent advances in understanding the biology of marginal zone lymphoma

Francesco Bertoni #  1   2 , Davide Rossi #  1   2 , Emanuele Zucca  1   2
Affiliations
Review

Recent advances in understanding the biology of marginal zone lymphoma

Francesco Bertoni et al. F1000Res. .

Abstract

There are three different marginal zone lymphomas (MZLs): the extranodal MZL of mucosa-associated lymphoid tissue (MALT) type (MALT lymphoma), the splenic MZL, and the nodal MZL. The three MZLs share common lesions and deregulated pathways but also present specific alterations that can be used for their differential diagnosis. Although trisomies of chromosomes 3 and 18, deletions at 6q23, deregulation of nuclear factor kappa B, and chromatin remodeling genes are frequent events in all of them, the three MZLs differ in the presence of recurrent translocations, mutations affecting the NOTCH pathway, and the transcription factor Kruppel like factor 2 ( KLF2) or the receptor-type protein tyrosine phosphatase delta ( PTPRD). Since a better understanding of the molecular events underlying each subtype may have practical relevance, this review summarizes the most recent and main advances in our understanding of the genetics and biology of MZLs.

Keywords: extranodal MZL of MALT type; genetics and biology of MZLs; marginal zone lymphoma; nodal MZL; splenic MZL.

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Conflict of interest statement

No competing interests were disclosed.No competing interests were disclosed.No competing interests were disclosed.

Figures

Figure 1.
Figure 1.. Summary of the main genetic and biologic features characterizing marginal zone lymphomas.
^Depending on the anatomical site. BCR, B-cell receptor; IGHV, immunoglobulin heavy variable; MALT, mucosa-associated lymphoid tissue; NF-κB, nuclear factor kappa B; NMZL, nodal marginal zone lymphoma; SMZL, splenic marginal zone lymphoma; TLR, Toll-like receptor.
See this image and copyright information in PMC

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Grants and funding

The author(s) declared that no grants were involved in supporting this work.

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