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Clinical and genetic features of pediatric acute lymphoblastic leukemia in Down syndrome in the Nordic countries

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Author

  • Catarina Lundin
  • Erik Forestier
  • Mette Klarskov Andersen
  • Kirsi Autio
  • Gisela Barbany
  • Lucia Cavelier
  • Irina Golovleva
  • Sverre Heim
  • Kristiina Heinonen
  • Randi Hovland
  • Johann H. Johannsson
  • Eigil Kjeldsen
  • Ann Nordgren
  • Lars Palmqvist
  • Bertil Johansson
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Summary, in English

Background: Children with Down syndrome (DS) have an increased risk for acute lymphoblastic leukemia (ALL). Although previous studies have shown that DS-ALL differs clinically and genetically from non-DS-ALL, much remains to be elucidated as regards genetic and prognostic factors in DS-ALL. Methods: To address clinical and genetic differences between DS-ALL and non-DS-ALL and to identify prognostic factors in DS-ALL, we ascertained and reviewed all 128 pediatric DS-ALL diagnosed in the Nordic countries between 1981 and 2010. Their clinical and genetic features were compared with those of the 4,647 B-cell precursor (BCP) ALL cases diagnosed during the same time period. Results: All 128 DS-ALL were BCP ALL, comprising 2.7% of all such cases. The 5-year event-free survival (EFS) and overall survival (OS) were significantly (P = 0.026 and P = 0.003, respectively) worse for DS-ALL patients with white blood cell counts >= 50 x 10(9)/l. The age distributions varied between the DS and non-DS cases, with age peaks at 2 and 3 years, respectively; none of the DS patients had infant ALL (P = 0.029). The platelet counts were lower in the DS-ALL group (P = 0.005). Abnormal karyotypes were more common in non-DS-ALL (P < 0.0001), and there was a significant difference in the modal number distribution, with only 2% high hyperdiploid DS-ALL cases (P < 0.0001). The 5-year EFS and 5-year OS were significantly worse for DS-ALL (0.574 and 0.691, respectively) compared with non-DS-ALL (0.783 and 0.894, respectively) in the NOPHO ALL-1992/2000 protocols (P < 0.001). Conclusions: The present study adds further support for genetic and clinical differences between DS-ALL and non-DS-ALL.

Department/s

Publishing year

2014

Language

English

Publication/Series

Journal of Hematology & Oncology

Volume

7

Document type

Journal article

Publisher

BioMed Central (BMC)

Topic

  • Medical Genetics

Keywords

  • Down syndrome
  • ALL
  • Children
  • Clinical features
  • Genetic features
  • Prognosis

Status

Published

Research group

  • Genetic and epigenetic studies of pediatric leukemia

ISBN/ISSN/Other

  • ISSN: 1756-8722