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AUTOIMMUNE AND INFLAMMATORY DISEASES ASSOCIATE TO R-IPSS SCORE BUT NOT TO OVERALL SURVIVAL OR LEUKEMIC EVOLUTION IN MYELODYSPLASTIC SYNDROMES

PF549

Brunet, T.1; Roy-Peaud, F.1; Landron, C.1; Gallego-Hernanz, M. P.1; Cayssials, E.1; Puyade, M.1; Torregrosa-Díaz, J. M.1

Poster Session I: Myelodysplastic syndromes - Clinical
Free

1CHU Poitiers, Poitiers, France

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Background:

Myelodysplastic syndromes (MDS) are clonal hemopathies with a relatively heterogeneous spectrum of presentation and a variable risk of transformation into acute myeloid leukemia (AML). Autoimmune diseases (AID) are present in 10 to 30% of MDS, with uncertain prognostic significance in this context. To date, no MDS-related factor has been clearly identified as a predictor for the occurrence of AIDs.

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Aims:

To study the association between MDS characteristics and the occurrence of an AID and its impact on overall survival and leukemia-free survival.

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Methods:

We retrospectively collected data from primary MDS adult patients followed-up at the University Hospital of Poitiers between January 2010 and December 2017. Immunosuppressive therapy secondary-MDS were excluded. The date of onset and type of AID as established by the standard criteria were collected. Steroid dependence was defined as a prednisone equivalent amount > 10 mg/day during at least 3 months. Simultaneous diagnosis of MDS and AID were considered if established in a 3-months interval from the MDS one. Baseline characteristics at diagnosis of the MDS were registered and compared between those with and without AIDs.

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Results:

AIDs were identified in 44 of 179 MDS patients (24.6%). MDS-AID patients were more frequently male (29/44, p < 0.05). AIDs occurred before MDS in 63.6% of cases. Associated AIDs were: systemic vasculitis (23%), inflammatory rheumatism (18%), neutrophilic dermatosis (12.5%), connective tissue diseases (12.5%) and autoimmune cytopenia (11%). At the time of AID diagnosis, 48% of patients had general signs, 37.5% had cutaneous and/or joint signs. AID was steroid-dependent in 39% of cases (9/23). No association between AID and MDS subtypes was observed. A significant linear tendency was found between the R-IPSS and the presence of AID (p = 0.02): that is, the lower the IPSS-R score, the higher the risk of association with AID. The presence of AID did not affect survival or progression to acute leukemia.

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Summary/Conclusion:

The association of MDS to AIDs is frequent and of clinical heterogeneous expression. It occurs mainly in low-risk MDS and does not seem to impact overall survival, that seems logical. The pathogenic mechanisms are complex and remain still completely unknown, but the hypothesis of a common pathogenic pathway seems strongly supported. The association MDS-AID does not seem to impact overall survival in our cohort and occurs mainly in low-risk MDS with a significant linear augmentation of the risk by the creasing categories of R-IPSS. That raises the question of the role of different physio-pathological processes underlying low-risk and high-risk MDS in the predisposition of AIDs. Larger prospective ongoing studies will better clarify our results.

Copyright © 2019 The Authors. Published by Wolters Kluwer Health Inc., on behalf of the European Hematology Association.