Zucca, Emanuele Rossi, Davide Bertoni, Francesco 2023 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. application/pdf https://n2t.net/ark:/12658/srd1326589 https://susi.usi.ch/global/documents/326589 https://susi.usi.ch/documents/326589/files/Zucca_2023_WILEY_hematoncology.pdf eng info:eu-repo/semantics/altIdentifier/doi/10.1002/hon.3152 info:eu-repo/semantics/altIdentifier/ark/12658/srd1326589 info:eu-repo/semantics/openAccess CC BY-NC-ND Hematological Oncology. - 91. - 2023, vol. 41, no. S1, p. 88 Extranodal MZL of MALT type Marginal zone lymphoma Nodal MZL Splenic MZL info:eu-repo/classification/udc/61 Marginal zone lymphomas http://purl.org/coar/resource_type/c_6501