Autoimmune manifestations associated with myelodysplastic syndromes

Eric Grignano (1, 2) , Vincent Jachiet (3, 4) , Pierre Fenaux (5) , Lionel Adès (5) , Olivier Fain (3, 4, 6) , Arsene Mekinian (3, 4, 6)

1 Hôpital Cochin [AP-HP]
2 AP-HP - Assistance publique - Hôpitaux de Paris (AP-HP)
3 CHU Saint-Antoine [AP-HP]
4 SU FM - Sorbonne Université - Faculté de Médecine
5 AP-HP - Hopital Saint-Louis [AP-HP]
6 CRSA - Centre de Recherche Saint-Antoine

Autoimmune disorders (ADs) are encountered in 10 to 20% of patients with myelodysplastic syndromes (MDS). Available data suggest that ADs concern more often younger patients with higher risk IPSS. MDS subtypes associated with ADs are mainly MDS with single lineage dysplasia (MDS-SLD) and MDS with excess blasts (MDS-EB). Various types of ADs have been described in association with MDS, ranging from limited clinical manifestations to systemic diseases affecting multiple organs. Defined clinical entities as vasculitis, connective tissue diseases, inflammatory arthritis, and neutrophilic diseases are frequently reported; however, unclassified or isolated organ impairment can be seen. In general, ADs do not seem to confer worse survival, although certain ADs may be associated with adverse outcomes (i.e., vasculitis) or progression of MDS (Sweet syndrome). While steroids and immunosuppressive treatment (IST) remain the backbone of first-line treatment, increasing evidence suggests that MDS-specific therapy as hypomethylating agents, based on their immunomodulatory effect, may be effective in treating these complications and for sparing steroids.

Mots clés: Myelodysplastic syndrome; Autoimmune disease; Hypomethylating agents

Domaines: Sciences du Vivant [q-bio]

Eric Grignano, Vincent Jachiet, Pierre Fenaux, Lionel Adès, Olivier Fain, et al.. Autoimmune manifestations associated with myelodysplastic syndromes. Annals of Hematology, 2018, 97 (11), pp.2015-2023. ⟨10.1007/s00277-018-3472-9⟩. ⟨hal-01960324⟩