Titre :

Inflammatory disorders associated with trisomy 8-myelodysplastic syndromes: French retrospective case-control study.

Auteur(s) :

Wesner, Nadege [Auteur]
Centre de Recherche Saint-Antoine [CRSA]
Drevon, Louis [Auteur]
Centre de Recherche Saint-Antoine [CRSA]
Guedon, Alexis [Auteur]
Institut Pierre Louis d'Epidémiologie et de Santé Publique [iPLESP]
Fraison, Jean-Baptiste [Auteur]
Trad, Salim [Auteur]
Kahn, Jean-Emmannuel [Auteur]
Lille Inflammation Research International Center - U 995 [LIRIC]
Aouba, Achille [Auteur]
Gillard, Jerome [Auteur]
Ponsoye, Matthieu [Auteur]
Hanslik, Thomas [Auteur]
Gourguechon, Clement [Auteur]
Liozon, Eric [Auteur]
Laribi, Kamel [Auteur]
Rossignol, Julien [Auteur]
Hermine, Olivier [Auteur]
Ades, Lionel [Auteur]
Carrat, Fabrice [Auteur]
Institut Pierre Louis d'Epidémiologie et de Santé Publique [iPLESP]
Fenaux, Pierre [Auteur]
Centre de Recherche Saint-Antoine [CRSA]
Mekinian, Arsene [Auteur]
Sorbonne Université [SU]
Fain, Olivier [Auteur]
Sorbonne Université [SU]

Titre de la revue :

European Journal of Haematology

Nom court de la revue :

Eur. J. Haematol.

Numéro :

102

Pagination :

63-69

Date de publication :

2019-01

ISSN :

1600-0609

Mot(s)-clé(s) :

immunology and infectious diseases
myelodysplastic syndromes

Discipline(s) HAL :

Sciences du Vivant [q-bio]

Résumé en anglais : [en]

Objective We report cases of myelodysplastic syndrome/myeloproliferative neoplasms (MDS/MPN) with trisomy 8 associated with inflammatory and autoimmune diseases (IADs). Method Data for 21 patients with trisomy 8-MDS/MPN ...
Lire la suite >Objective We report cases of myelodysplastic syndrome/myeloproliferative neoplasms (MDS/MPN) with trisomy 8 associated with inflammatory and autoimmune diseases (IADs). Method Data for 21 patients with trisomy 8-MDS/MPN and IADs were analyzed and compared to 103 patients with trisomy 8-MDS/MPN without IADs. Results The median age of MDS/MPN patients with IADs was 67 [59-80]. The IADs were Behçet's-like disease in 11 (52%) patients, inflammatory arthritis in 4 (19%) and Sjögren's syndrome, autoimmune hemolytic anemia, aseptic abscess, periarteritis nodosa, Sweet's syndrome and unclassified vasculitis in one patient each. Overall, 17/21 (81%) patients with IADs received treatment (88% with steroids), with complete and partial response in 7/17 (35%) and 8/17 (47%), respectively. The effect of MDS treatment on IADs could be assessed in seven patients receiving azacytidine: five achieved remission and two partial response. As compared with the 103 trisomy 8-MDS/MPN cases without IADs, those with IADs were more often non-European (P = 0.005) and had poor karyotype (P < 0.001). We found no difference in overall survival or acute myeloid leukemia progression between trisomy 8-associated MDS/MPN with and without IADs. Conclusion The spectrum of IADs associated with trisomy 8-positive MDS/MPN is dominated by Behçet's-like disease. Steroid therapy is effective, but mostly sparing therapies are necessary. Azacytidine could be an effective alternative.Lire moins >

Langue :

Anglais

Audience :

Internationale

Vulgarisation :

Non

Établissement(s) :

Inserm
Université de Lille
CHU Lille

Collections :

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

Équipe(s) de recherche :

Immunity, inflammation and fibrsis in auto and allo-reactivity

Date de dépôt :

2019-03-01T14:35:23Z
2024-03-07T16:41:29Z