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Article Dans Une Revue Expert Review of Clinical Immunology Année : 2024

The complex relationship between large granular lymphocyte leukemia and rheumatic disease

Résumé

Introduction: Large granular lymphocytic (LGL) leukemia is a rare lymphoproliferative disorder characterized by an expansion of clonal T or NK lymphocytes. Neutropenia-related infections represent the main clinical manifestation. Even if the disease follows an indolent course, most patients will ultimately need treatment in their lifetime. Interestingly, LGL leukemia is characterized by a high frequency of autoimmune disorders with rheumatoid arthritis being the most frequent.Areas covered: This review covers the pathophysiology, clinic-biological features and the advances made in the treatment of LGL leukemia. A special focus will be made on the similarities in the pathophysiology of LGL leukemia and the frequently associated rheumatic disorders.Expert opinion: Recent advances in the phenotypic and molecular characterization of LGL clones have uncovered the key role of JAK-STAT signaling in the pathophysiology linking leukemic cells expansion and autoimmunity. The description of the molecular landscape of T- and NK-LGL leukemia and the improved understanding of the associated rheumatic disorders open the way to the development of new targeted therapies effective on both conditions.
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hal-04382900 , version 1 (09-01-2024)

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Tony Marchand, Thierry Lamy. The complex relationship between large granular lymphocyte leukemia and rheumatic disease. Expert Review of Clinical Immunology, 2024, Expert Review of Clinical Immunology, 20 (3), pp.291-303. ⟨10.1080/1744666x.2023.2292758⟩. ⟨hal-04382900⟩
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