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

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  • 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
  • Nordic Society of Pediatric Hematology Oncology (NOPHO)
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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 × 109/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.

Original languageEnglish
JournalJournal of Hematology & Oncology
Pages (from-to)32
Publication statusPublished - 2014

    Research areas

  • Adolescent, Child, Child, Preschool, Chromosome Aberrations, Chromosome Banding, Denmark, Disease-Free Survival, Down Syndrome, Female, Finland, Humans, Iceland, In Situ Hybridization, Fluorescence, Infant, Karyotype, Karyotyping, Leukocyte Count, Male, Norway, Platelet Count, Precursor Cell Lymphoblastic Leukemia-Lymphoma, Registries, Sweden, Treatment Outcome

ID: 45088583