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

Autor: Wesner N; Department of Internal Medicine, Inflammation-Immunopathology-Biotherapy Department (DHU i2B), Assistance Publique-Hôpitaux de Paris, Hôpital Saint-Antoine, Paris, France.; Sorbonne Universités, F-75012 Paris, France.; INSERM U938, Centre de Recherche Saint-Antoine (CRSA), UMPC University Paris 06, Sorbonne Universités, Paris, France., Drevon L; Department of Internal Medicine, Inflammation-Immunopathology-Biotherapy Department (DHU i2B), Assistance Publique-Hôpitaux de Paris, Hôpital Saint-Antoine, Paris, France.; Sorbonne Universités, F-75012 Paris, France.; INSERM U938, Centre de Recherche Saint-Antoine (CRSA), UMPC University Paris 06, Sorbonne Universités, Paris, France., Guedon A; INSERM, Institut Pierre Louis d'Epidémiologie et de Santé Publique IPLESP, AP-HP, Hôpital Saint Antoine, Sorbonne Université, Paris, France., Fraison JB; Department of Internal Medicine, Hôpital Sète, Sète, France., Trad S; Department of Internal Medicine, Assistance Publique-Hôpitaux de Paris, Hôpital Ambroise Paré, Boulogne Billancourt, France., Kahn JE; Department of Internal Medicine, Hôpital Foch, Suresnes, France., Aouba A; Department of Internal Medicine, CH Caen, Caen, France., Gillard J; Department of Rheumatology, CH Lons le Saunier, Lons le Saunier, France., Ponsoye M; Department of Internal Medicine, Assistance Publique-Hôpitaux de Paris, Hôpital Ambroise Paré, Boulogne Billancourt, France., Hanslik T; Department of Internal Medicine, Assistance Publique-Hôpitaux de Paris, Hôpital Ambroise Paré, Boulogne Billancourt, France., Gourguechon C; Department of Internal Medicine, CHU Amiens-Picardie, Amiens, France., Liozon E; Department of Internal Medicine, CHU Limoges, Limoges, France., Laribi K; Department of Hematology, Centre Hospitalier Le Mans, Le Mans, France., Rossignol J; Department of Hematology, Assistance Publique-Hôpitaux de Paris, Hôpital Necker Enfants Malades, Paris, France., Hermine O; Department of Hematology, Assistance Publique-Hôpitaux de Paris, Hôpital Necker Enfants Malades, Paris, France., Adès L; Department of Hematology, Assistance Publique-Hôpitaux de Paris, Hôpital Saint Louis, Paris, France., Carrat F; INSERM, Institut Pierre Louis d'Epidémiologie et de Santé Publique IPLESP, AP-HP, Hôpital Saint Antoine, Sorbonne Université, Paris, France., Fenaux P; INSERM U938, Centre de Recherche Saint-Antoine (CRSA), UMPC University Paris 06, Sorbonne Universités, Paris, France., Mekinian A; Department of Internal Medicine, Inflammation-Immunopathology-Biotherapy Department (DHU i2B), Assistance Publique-Hôpitaux de Paris, Hôpital Saint-Antoine, Paris, France.; Sorbonne Universités, F-75012 Paris, France., Fain O; Department of Internal Medicine, Inflammation-Immunopathology-Biotherapy Department (DHU i2B), Assistance Publique-Hôpitaux de Paris, Hôpital Saint-Antoine, Paris, France.; Sorbonne Universités, F-75012 Paris, France.
Jazyk: angličtina
Zdroj: European journal of haematology [Eur J Haematol] 2019 Jan; Vol. 102 (1), pp. 63-69. Date of Electronic Publication: 2018 Nov 14.
DOI: 10.1111/ejh.13174
Abstrakt: 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.
(© 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)
Databáze: MEDLINE
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