T(6;9)(p22;q34)/DEK-NUP214-rearranged pediatric myeloid leukemia: An international study of 62 patients

Julie Damgaard Sandahl, Eva A. Coenen, Erik Forestier, Jochen Harbott, Bertil Johansson, Gitte Kerndrup, Souichi Adachi, Anne Auvrignon, H. Berna Beverloo, Jean Michel Cayuela, Lucy Chilton, Maarten Fornerod, Valérie de Haas, Christine J. Harrison, Hiroto Inaba, Gertjan J.L. Kaspers, Der Cherng Liang, Franco Locatelli, Riccardo Masetti, Christine PerotSusana C. Raimondi, Katarina Reinhardt, Daisuke Tomizawa, Nils von Neuhoff, Marco Zecca, C. Michel Zwaan, Marry M. van den Heuvel-Eibrink, Henrik Hasle

Onderzoeksoutput: Bijdrage aan tijdschriftArtikelpeer review

65 Citaten (Scopus)

Samenvatting

Acute myeloid leukemia with t(6;9)(p22;q34) is listed as a distinct entity in the 2008 World Health Organization classification, but little is known about the clinical implications of t(6;9)-positive myeloid leukemia in children. This international multicenter study presents the clinical and genetic characteristics of 62 pediatric patients with t(6;9)/DEK-NUP214-rearranged myeloid leukemia; 54 diagnosed as having acute myeloid leukemia, representing <1% of all childhood acute myeloid leukemia, and eight as having myelodysplastic syndrome. The t(6;9)/DEK-NUP214 was associated with relatively late onset (median age 10.4 years), male predominance (sex ratio 1.7), French-American-British M2 classification (54%), myelodysplasia (100%), and FLT3-ITD (42%). Outcome was substantially better than previously reported with a 5-year event-free survival of 32%, 5-year overall survival of 53%, and a 5-year cumulative incidence of relapse of 57%. Hematopoietic stem cell transplantation in first complete remission improved the 5-year event-free survival compared with chemotherapy alone (68% versus 18%; P<0.01) but not the overall survival (68% versus 54%; P=0.48). The presence of FLT3-ITD had a non-significant negative effect on 5-year overall survival compared with non-mutated cases (22% versus 62%; P=0.13). Gene expression profiling showed a unique signature characterized by significantly higher expression of EYA3, SESN1, PRDM2/RIZ, and HIST2H4 genes. In conclusion, t(6;9)/DEK-NUP214 represents a unique subtype of acute myeloid leukemia with a high risk of relapse, high frequency of FLT3-ITD, and a specific gene expression signature.

Originele taal-2Engels
Pagina's (van-tot)865-872
Aantal pagina's8
TijdschriftHaematologica
Volume99
Nummer van het tijdschrift5
DOI's
StatusGepubliceerd - 1 mei 2014
Extern gepubliceerdJa

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