1. [Dysimmune manifestations associated with myelodysplastic neoplasms and chronic myelomonocytic leukaemias].
- Author
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Jachiet V, Hadjadj J, Zhao LP, Chasset F, Fain O, Fenaux P, and Mekinian A
- Subjects
- Humans, Azacitidine therapeutic use, Leukemia, Myelomonocytic, Chronic complications, Leukemia, Myelomonocytic, Chronic genetics, Leukemia, Myelomonocytic, Chronic drug therapy, Myelodysplastic Syndromes complications, Myelodysplastic Syndromes genetics, Skin Diseases
- Abstract
Systemic inflammatory or autoimmune diseases (SIAD) are observed in up to a quarter of patients with myelodysplastic syndromes (MDS) or chronic myelomonocytic leukemia (CMML), with a broad clinical spectrum including asymptomatic biological abnormalities, isolated inflammatory clinical manifestations (recurrent fever, arthralgia, neutrophilic dermatoses…) or identified systemic diseases (giant cell arteritis, recurrent polychondritis…). Recent advances in molecular biology have shed new light on the pathophysiological mechanisms that link inflammatory manifestations and myeloid hemopathies, particularly in VEXAS syndrome following the identification of somatic mutations in the UBA1 gene, or in neutrophilic dermatoses with the concept of myelodysplasia cutis. Although the presence of SIAD does not seem to affect overall survival or the risk of transformation into acute myeloid leukemia, their treatment remains a challenge given the frequent high level of corticosteroid dependence as well as the poor efficacy and tolerance (cytopenias, infections) of conventional immunosuppressive agents. Recent prospective data supports the interest of a therapeutic strategy using demethylating agents and notably azacitidine to target the pathological clone., (Copyright © 2023 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.) more...
- Published
- 2023
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