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

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Author

  • 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
  • Valerie de Haas
  • Christine J. Harrison
  • Hiroto Inaba
  • Gertjan J. L. Kaspers
  • Der-Cherng Liang
  • Franco Locatelli
  • Riccardo Masetti
  • Christine Perot
  • Susana C. Raimondi
  • Katarina Reinhardt
  • Daisuke Tomizawa
  • Nils von Neuhoff
  • Marco Zecca
  • C. Michel Zwaan
  • Marry M. van den Heuvel-Eibrink
  • Henrik Hasle
Show all

Summary, in English

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)/DEKNUP214-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.

Department/s

Publishing year

2014

Language

English

Pages

865-872

Publication/Series

Haematologica

Volume

99

Issue

5

Document type

Journal article

Publisher

Ferrata Storti Foundation

Topic

  • Hematology

Status

Published

ISBN/ISSN/Other

  • ISSN: 1592-8721