AUTOIMMUNE AND INFLAMMATORY DISEASES ASSOCIATE TO R-IPSS SCORE BUT NOT TO OVERALL SURVIVAL OR LEUKEMIC EVOLUTION IN MYELODYSPLASTIC SYNDROMES
Author(s): ,
Thomas Brunet
Affiliations:
CHU Poitiers,Poitiers,France
,
Frederique Roy-Peaud
Affiliations:
CHU Poitiers,Poitiers,France
,
Cedric Landron
Affiliations:
CHU Poitiers,Poitiers,France
,
Maria Pilar Gallego-Hernanz
Affiliations:
CHU Poitiers,Poitiers,France
,
Emilie Cayssials
Affiliations:
CHU Poitiers,Poitiers,France
,
Mathieu Puyade
Affiliations:
CHU Poitiers,Poitiers,France
Jose Miguel Torregrosa-Díaz
Affiliations:
CHU Poitiers,Poitiers,France
EHA Library. Brunet T. Jun 14, 2019; 266349; PF549
Thomas Brunet
Thomas Brunet
Contributions
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Abstract

Abstract: PF549

Type: Poster Presentation

Presentation during EHA24: On Friday, June 14, 2019 from 17:30 - 19:00

Location: Poster area

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.

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

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.

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.

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.

Session topic: 10. Myelodysplastic syndromes - Clinical

Keyword(s): Autoimmune disease, Myelodysplasia

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