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Autoimmune manifestations associated with myelodysplastic syndromes

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Abstract

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.

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Authors and Affiliations

  1. Department of Hematology, Assistance Publique–Hôpitaux de Paris, Hôpital Cochin, 75014, Paris, France

    Eric Grignano

  2. Department of Internal Medicine, Inflammation-Immunopathology-Biotherapy Department (DHU i2B), Assistance Publique - Hôpitaux de Paris, Hôpital Saint-Antoine, 184, rue du Faubourg Saint-Antoine, 75012, Paris, France

    Vincent Jachiet, Olivier Fain & Arsène Mekinian

  3. UPMC University Paris 06, Paris, France

    Vincent Jachiet, Olivier Fain & Arsène Mekinian

  4. Department of Hematology, Assistance Publique–Hôpitaux de Paris, Hôpital Saint Louis, 1 avenue Claude Vellefaux, 75010, Paris, France

    Pierre Fenaux & Lionel Ades

  5. Sorbonne Universités, UMPC University Paris 06, INSERM U938, Centre de Recherche Saint-Antoine (CRSA), Paris, France

    Olivier Fain & Arsène Mekinian

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• EG performed the research

• EG and AM designed the research study

• VJ and AM contributed essential reagents or tools

• PF, LA and OF revised the manuscript.

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Correspondence to Arsène Mekinian.

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Grignano, E., Jachiet, V., Fenaux, P. et al. Autoimmune manifestations associated with myelodysplastic syndromes. Ann Hematol 97, 2015–2023 (2018). https://doi.org/10.1007/s00277-018-3472-9

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