Abstract:
:Isolated trisomy 8 (+8) is a frequent cytogenetic abnormality in the myelodysplastic syndromes (MDS), but its characteristics are poorly reported. We performed a retrospective study of 138 MDS patients with isolated +8, classified or reclassified as MDS (excluding MDS/myeloproliferative neoplasm). Myeloproliferative (MP) features were defined by the repeated presence of one of the following: white blood cell count >10 × 109 /l, myelemia (presence of circulating immature granulocytes with a predominance of more mature forms) >2%, palpable splenomegaly. Fifty-four patients (39·1%) had MP features: 28 at diagnosis, 26 were acquired during evolution. MP forms had more EZH2 (33·3% vs. 12·0% in non-MP, P = 0·047), ASXL1 (66·7% vs. 42·3%, P = 0·048) and STAG2 mutations (77·8% vs. 21·7%, P = 0·006). Median event-free survival (EFS) and overall survival (OS) were 25 and 27 months for patients with MP features at diagnosis, versus 28 (P = 0·15) and 39 months (P = 0·085) for those without MP features, respectively. Among the 57 patients who received hypomethylating agent (HMA), OS was lower in MP cases (13 months vs. 23 months in non-MP cases, P = 0.02). In conclusion, MP features are frequent in MDS with isolated +8. MP forms had more EZH2, ASXL1 and STAG2 mutations, responded poorly to HMA, and tended to have poorer survival than non-MP forms.
journal_name
Br J Haematoljournal_title
British journal of haematologyauthors
Drevon L,Marceau A,Maarek O,Cuccuini W,Clappier E,Eclache V,Cluzeau T,Richez V,Berkaoui I,Dimicoli-Salazar S,Bidet A,Vial JP,Park S,Vieira Dos Santos C,Kaphan E,Berthon C,Stamatoullas A,Delhommeau F,Abermil N,Braundoi
10.1111/bjh.15490subject
Has Abstractpub_date
2018-09-01 00:00:00pages
843-850issue
6eissn
0007-1048issn
1365-2141journal_volume
182pub_type
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