Title :

Reduced peripheral blood dendritic cell and monocyte subsets in MDS patients with systemic inflammatory or dysimmune diseases.

Author(s) :

Jachiet, Vincent [Auteur]
Centre de Recherche Saint-Antoine [CRSA]
Ricard, Laure [Auteur]
Centre de Recherche Saint-Antoine [CRSA]
Hirsch, Pierre [Auteur]
CHU Saint-Antoine [AP-HP]
Malard, Florent [Auteur]
Centre de Recherche Saint-Antoine [CRSA]
Pascal, Laurent [Auteur]
Hôpital Saint Vincent de Paul de Lille
Beyne-Rauzy, Odile [Auteur]
Institut Universitaire du Cancer de Toulouse - Oncopole [IUCT Oncopole - UMR 1037]
Peterlin, Pierre [Auteur]
Centre Hospitalier Universitaire de Nantes = Nantes University Hospital [CHU Nantes]
Maria, Alexandre T. J. [Auteur]
Hôpital Saint Eloi [CHU Montpellier]
Vey, Norbert [Auteur]
Centre de Recherche en Cancérologie de Marseille [CRCM]
D'aveni, Maud [Auteur]
Centre Hospitalier Régional Universitaire de Nancy [CHRU Nancy]
Gourin, Marie-Pierre [Auteur]
Hôpital Dupuytren [CHU Limoges]
Dimicoli-Salazar, Sophie [Auteur]
Hôpital Haut-Lévêque [CHU Bordeaux]
Banos, Anne [Auteur]
Centre Hospitalier de la Côte Basque [CHCB]
Terriou, Louis [Auteur]
Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286
De Renzis, Benoit [Auteur]
CHU Estaing [Clermont-Ferrand]
Durot, Eric [Auteur]
Hôpital universitaire Robert Debré [Reims] [CHU Reims]
Natarajan-Ame, Shanti [Auteur]
Institut de Cancérologie de Strasbourg Europe [ICANS]
Vekhoff, Anne [Auteur]
CHU Saint-Antoine [AP-HP]
Voillat, Laurent [Auteur]
Centre Hospitalier Chalon-sur-Saône William Morey
Park, Sophie [Auteur]
Centre Hospitalier Universitaire [CHU Grenoble] [CHUGA]
Vinit, Julien [Auteur]
Centre Hospitalier Chalon-sur-Saône William Morey
Dieval, Céline [Auteur]
Centre Hospitalier de Rochefort [CH Rochefort]
Dellal, Azeddine [Auteur]
Groupe Hospitalier Intercommunal Le Raincy-Montfermeil

Journal title :

Clinical and Experimental Medicine

Abbreviated title :

Clin Exp Med

Volume number :

23

Pages :

803–813

Publication date :

2022-08-15

ISSN :

1591-9528

English keyword(s) :

Myelodysplastic syndrome
VEXAS syndrome
Inflammatory disease
Dendritic cells
Monocytes

HAL domain(s) :

Sciences du Vivant [q-bio]

English abstract : [en]

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 ...
Show more >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.Show less >

Language :

Anglais

Audience :

Internationale

Popular science :

Non

Administrative institution(s) :

Université de Lille
Inserm
CHU Lille

Collections :

• Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286

Submission date :

2024-01-12T00:52:29Z
2024-03-28T07:56:17Z