High incidence of the ETV6/RUNX1 fusion gene in paediatric precursor B-cell acute lymphoblastic leukaemias with trisomy 21 as the sole cytogenetic change: a Nordic series of cases diagnosed 1989-2005

Br J Haematol. 2006 Nov;135(3):352-4. doi: 10.1111/j.1365-2141.2006.06286.x. Epub 2006 Sep 11.

Abstract

Trisomy 21 is common in ETV6/RUNX1-positive acute lymphoblastic leukaemia (ALL); both these aberrations are associated with a favourable outcome. The prognostic impact of +21 as a sole cytogenetic change could be due to a cryptic t(12;21)(p13;q22). The occurrence of ETV6/RUNX1 was determined in 66 childhood ALLs with an acquired +21 and a chromosome number <51. ETV6/RUNX1 was found in 45% of cases and in the majority (10/18; 56%) of ALLs with sole +21. Event-free survival did not differ between the t(12;21)-positive and -negative cases. Thus, the prognostic impact of +21 is not attributable to cryptic ETV6/RUNX1.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Burkitt Lymphoma / epidemiology
  • Burkitt Lymphoma / genetics*
  • Child
  • Child, Preschool
  • Chromosome Aberrations
  • Chromosomes, Human, Pair 21 / genetics*
  • Core Binding Factor Alpha 2 Subunit / genetics*
  • ETS Translocation Variant 6 Protein
  • Female
  • Finland / epidemiology
  • Genes, Neoplasm / genetics*
  • Humans
  • Iceland / epidemiology
  • Incidence
  • Infant
  • Male
  • Neoplasm Proteins / genetics*
  • Prognosis
  • Proto-Oncogene Proteins c-ets / genetics*
  • Repressor Proteins / genetics*
  • Scandinavian and Nordic Countries / epidemiology

Substances

  • Core Binding Factor Alpha 2 Subunit
  • Neoplasm Proteins
  • Proto-Oncogene Proteins c-ets
  • RUNX1 protein, human
  • Repressor Proteins