In:
European Journal of Haematology, Wiley, Vol. 102, No. 1 ( 2019-01), p. 63-69
Abstract:
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.
Type of Medium:
Online Resource
ISSN:
0902-4441
,
1600-0609
DOI:
10.1111/ejh.2019.102.issue-1
Language:
English
Publisher:
Wiley
Publication Date:
2019
detail.hit.zdb_id:
2027114-1
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