Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2023 Jun:41 Suppl 1:88-91.
doi: 10.1002/hon.3152.

Marginal zone lymphomas

Emanuele Zucca  1   2   3 , Davide Rossi  1   2   3 , Francesco Bertoni  1   2   3
Affiliations
Review

Marginal zone lymphomas

Emanuele Zucca et al. Hematol Oncol. 2023 Jun.

Abstract

The three main types of marginal zone lymphoma (MZL), recognized by the current lymphoma classifications are the extranodal MZL of mucosa-associated lymphoid tissue, the splenic MZL, and the nodal MZL. They share some karyotype lesions (trisomies of chromosomes 3 and 18, deletions at 6q23), and alterations of the nuclear factor kappa B (NFkB) pathway are also common in all of them. However, they differ in the presence of recurrent translocations, mutations affecting the Notch signaling pathway (NOTCH2 and less commonly NOTCH1), the transcription factors Kruppel-like factor 2 (KLF2) or the receptor-type protein tyrosine phosphatase delta (PTPRD). This review summarizes the most recent and significant advances in our understanding of the epidemiology, genetics, and biology of MZLs and outlines the current principles of the standard management of MZL at different anatomic sites.

Keywords: extranodal MZL of MALT type; marginal zone lymphoma; nodal MZL; splenic MZL.

PubMed Disclaimer

References

REFERENCES

    1. Rossi D, Bertoni F, Zucca E. Marginal-zone lymphomas. N Engl J Med. 2022;386(6):568-581. https://doi.org/10.1056/nejmra2102568
    1. Campo E, Jaffe ES, Cook JR, et al. The International Consensus Classification of mature lymphoid neoplasms: a report from the Clinical Advisory Committee. Blood. 2022;140(11):1229-1253. https://doi.org/10.1182/blood.2022015851
    1. Alaggio R, Amador C, Anagnostopoulos I, et al. The 5th edition of the World Health Organization classification of haematolymphoid tumours: lymphoid neoplasms. Leukemia. 2022;36(7):1720-1748. https://doi.org/10.1038/s41375-022-01620-2
    1. Palm A.-KE, Kleinau S. Marginal zone B cells: from housekeeping function to autoimmunity? J Autoimmun. 2021;119:102627. https://doi.org/10.1016/j.jaut.2021.102627
    1. Song H, Cerny J. Functional heterogeneity of marginal zone B cells revealed by their ability to generate both early antibody-forming cells and germinal centers with hypermutation and memory in response to a T-dependent antigen. J Exp Med. 2003;198(12):1923-1935. https://doi.org/10.1084/jem.20031498

MeSH terms

Substances

LinkOut - more resources