Wesner N, Drevon L, Guedon A, Fraison JB, Trad S, Kahn JE, Aouba A, Gillard J, Ponsoye M, Hanslik T, Gourguechon C, Liozon E, Laribi K, Rossignol J, Hermine O, Adès L, Carrat F, Fenaux P, Mekinian A, Fain O; GFM, MINHEMON (French Network of Dysimmune Disorders Associated to Hemopathies).
Eur J Haematol. 2019 Jan;102(1):63-69. doi: 10.1111/ejh.13174. Epub 2018 Nov 14.
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.https://www.ncbi.nlm.nih.gov/pubmed/30218579