Reduced peripheral blood dendritic cell and monocyte subsets in MDS patients with systemic inflammatory or dysimmune diseases.
Fiche publication
Date publication
août 2022
Journal
Clinical and experimental medicine
Auteurs
Membres identifiés du Cancéropôle Est :
Dr AME Shanti, Dr D'AVENI-PINEY Maud
Tous les auteurs :
Jachiet V, Ricard L, Hirsch P, Malard F, Pascal L, Beyne-Rauzy O, Peterlin P, Maria ATJ, Vey N, D'Aveni M, Gourin MP, Dimicoli-Salazar S, Banos A, Wickenhauser S, Terriou L, De Renzis B, Durot E, Natarajan-Ame S, Vekhoff A, Voillat L, Park S, Vinit J, Dieval C, Dellal A, Grobost V, Willems L, Rossignol J, Solary E, Kosmider O, Dulphy N, Zhao LP, Adès L, Fenaux P, Fain O, Mohty M, Gaugler B, Mekinian A,
Lien Pubmed
Résumé
Systemic inflammatory and autoimmune diseases (SIADs) occur in 10-20% of patients with myelodysplastic syndrome (MDS). Recently identified VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) syndrome, associated with somatic mutations in UBA1 (Ubiquitin-like modifier-activating enzyme 1), encompasses a range of severe inflammatory conditions along with hematological abnormalities, including MDS. The pathophysiological mechanisms underlying the association between MDS and SIADs remain largely unknown, especially the roles of different myeloid immune cell subsets. The aim of this study was to quantitatively evaluate peripheral blood myeloid immune cells (dendritic cells (DC) and monocytes) by flow cytometry in MDS patients with associated SIAD (n = 14, most often including relapsing polychondritis or neutrophilic dermatoses) and to compare their distribution in MDS patients without SIAD (n = 23) and healthy controls (n = 7). Most MDS and MDS/SIAD patients had low-risk MDS. Eight of 14 (57%) MDS/SIAD patients carried UBA1 somatic mutations, defining VEXAS syndrome.Compared with MDS patients, most DC and monocyte subsets were significantly decreased in MDS/SIAD patients, especially in MDS patients with VEXAS syndrome. Our study provides the first overview of the peripheral blood immune myeloid cell distribution in MDS patients with associated SIADs and raises several hypotheses: possible redistribution to inflammation sites, increased apoptosis, or impaired development in the bone marrow.
Mots clés
Dendritic cells, Inflammatory disease, Monocytes, Myelodysplastic syndrome, VEXAS syndrome
Référence
Clin Exp Med. 2022 08 11;: