• N Wesner, P Fenaux, V Jachiet, L Ades, O Fain, A Mekinian, and MINHEMON (French Network of dysimmune disorders associated with hemopathies).
• Department of internal medicine, inflammation-immunopathology-biotherapy department (DMU I3), Assistance publique-Hôpitaux de Paris, hôpital Saint-Antoine, Sorbonne université, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France; Sorbonne universités, Inserm U938, centre de recherche Saint-Antoine (CRSA), Paris, France.
• Rev Med Interne. 2021 Mar 1; 42 (3): 170-176.

AbstractMyelodysplastic syndromes (MDS) are clonal hematopoietic malignancies which are also characterised by immune dysregulation. The impaired immune response is mainly due to T lymphocytes (CD8 and T regulatory cells) with increased cell apoptosis. MDS could be associated in some cases with various clinical dysimmune features; however, only MDS with trisomy 8 is correlated with particular clinical phenotype. The latter is mainly Behçet's-like disease which includes orogenital aphtosis, skin features and severe ulcerative digestive disease of ileocaecal distribution. Other clinical manifestations, such as arthritis or neutrophilic dermatosis, have been also described in MDS patients with trisomy 8. The dysimmune manifestations, and among them the Behçet's-like disease, do not impact the overall survival or the risk of progression to acute myeloid leukemia. Immunosuppressive and immunomodulatory therapies, and among them TNF-α inhibitors, are usually ineffective to control the dysimmune manifestations. Targeting the underlying clonal disease with specific therapies, such as azacitidine, seems to be the best strategy to control these disorders, even in MDS patients with low-risk disease.Copyright © 2020 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.

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