Mirandari, Amatta, Parker, Helen, Ashton-Key, Margaret, Stevens, Benjamin, Walewska, Renata, Stamatopoulos, Kostas, Bryant, Dean, Oscier, David G., Gibson, Jane and Strefford, Jonathan C. (2024) The genomic and molecular landscape of splenic marginal zone lymphoma, biological and clinical implications. Exploration of Targeted Anti-Tumor Therapy, 5 (5), 877-901. (doi:10.37349/etat.2024.00253).
Abstract
Splenic marginal zone lymphoma (SMZL) is a rare, predominantly indolent B-cell lymphoma constituting fewer than 2% of lymphoid neoplasms. However, around 30% of patients have a shorter survival despite currently available treatments and the prognosis is especially poor for the 5–15% of cases that transform to a large cell lymphoma. Mounting evidence suggests that the molecular pathogenesis of SMZL is critically shaped by microenvironmental triggering and cell-intrinsic aberrations. Immunogenetic investigations have revealed biases in the immunoglobulin gene repertoire, indicating a role of antigen selection. Furthermore, cytogenetic studies have identified recurrent chromosomal abnormalities such as deletion of the long arm of chromosome 7, though specific disease-associated genes remain elusive. Our knowledge of SMZL’s mutational landscape, based on a limited number of cases, has identified recurring mutations in KLF2, NOTCH2, and TP53, as well as genes clustering within vital B-cell differentiation pathways
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