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Zucca_2023_WILEY_hematoncology
Journal article

Marginal zone lymphomas

  • Zucca, Emanuele Oncology Institute of Southern Switzerland (IOSI), Bellinzona, Switzerland - Institute of Oncology Research (IOR), Faculty of Biomedical Sciences, Università della Svizzera italiana, Switzerland - Faculty of Biomedical Sciences, Università della Svizzera italiana, Switzerland
  • Rossi, Davide Oncology Institute of Southern Switzerland (IOSI), Bellinzona, Switzerland - Institute of Oncology Research (IOR), Faculty of Biomedical Sciences, Università della Svizzera italiana, Switzerland - Faculty of Biomedical Sciences, Università della Svizzera italiana, Switzerland
  • Bertoni, Francesco Oncology Institute of Southern Switzerland (IOSI), Bellinzona, Switzerland - Institute of Oncology Research (IOR), Faculty of Biomedical Sciences, Università della Svizzera italiana, Switzerland - Faculty of Biomedical Sciences, Università della Svizzera italiana, Switzerland
  • 2023
Published in:
  • Hematological Oncology. - 91. - 2023, vol. 41, no. S1, p. 88
Extranodal MZL of MALT type
Marginal zone lymphoma
Nodal MZL
Splenic MZL
English 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.
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  • English
Classification
Medicine
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CC BY-NC-ND
Open access status
green
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https://n2t.net/ark:/12658/srd1326589
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