Article
Reduced peripheral blood dendritic cell and monocyte subsets in MDS patients with systemic inflammatory or dysimmune diseases.
Clinical and experimental medicine - 1 Jul 2023
Jachiet Vincent, Ricard Laure, Hirsch Pierre, Malard Florent, Pascal Laurent, Beyne-Rauzy Odile, Peterlin Pierre, Maria Alexandre Thibault Jacques, Vey Norbert, D'Aveni Maud, Gourin Marie-Pierre, Dimicoli-Salazar Sophie, Banos Anne, Wickenhauser Stefan, Terriou Louis, De Renzis Benoit, Durot Eric, Natarajan-Ame Shanti, Vekhoff Anne, Voillat Laurent, Park Sophie, Vinit Julien, Dieval Céline, Dellal Azeddine, Grobost Vincent, Willems Lise, Rossignol Julien, Solary Eric, Kosmider Olivier, Dulphy Nicolas, Zhao Lin Pierre, Adès Lionel, Fenaux Pierre, Fain Olivier, Mohty Mohamad, Gaugler Béatrice, Mekinian Arsène
Abstract excerpt
BACKGROUND: 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,...
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