000 02290cam a2200205 4500500
005 20251012013150.0
041 _afre
042 _adc
100 1 0 _aFrankowski, Enzo
_eauthor
700 1 0 _a Hervouet, Louise
_eauthor
700 1 0 _a Papin, Antonin
_eauthor
700 1 0 _a Tessoulin, Benoît
_eauthor
700 1 0 _a Chiron, David
_eauthor
245 0 0 _aAdvancing the biological understanding of mantle cell lymphoma to drive therapeutic innovation
260 _c2025.
500 _a71
520 _aMantle cell lymphoma (MCL) is a rare and aggressive B-cell non-Hodgkin lymphoma, characterized by specific markers such as cyclin D1 and SOX11, along with a well-defined mutational profile. More recently, studies have highlighted the central role of the tumor microenvironment in disease progression and the development of therapeutic resistance, through complex interactions within protective ecosystems. These major advances in the understanding of MCL biology, combined with optimized clinical protocols, have led to a paradigm shift, with prolonged remissions now achievable in some patients following immunochemotherapy. However, the toxicity of such treatments limits their use in frail patients, and a significant proportion of high-risk, refractory cases still lack effective therapeutic options. In this context, the rapid emergence of targeted therapies and immunotherapeutic approaches offers new perspectives by counteracting malignant plasticity and intratumoral heterogeneity. This review first provides an overview of current standards in MCL management, then explores how both intrinsic tumor alterations and interactions with the microenvironment contribute to resistance mechanisms. We then present recent strategies aimed at targeting tumor vulnerabilities within its protective niches. Finally, we discuss the potential of emerging approaches — such as the modulation of epigenetic dysregulation and the restoration of functional lymphoid immunity through modern immunotherapies — as a foundation for future therapeutic options for patients currently facing treatment failure.
786 0 _nHématologie | 31 | 4 | 2025-09-17 | p. 287-303 | 1264-7527
856 4 1 _uhttps://shs.cairn.info/journal-hematologie-2025-4-page-287?lang=en&redirect-ssocas=7080
999 _c1528604
_d1528604