Haematologica (Nov 2007)

Incidence and outcome of TCF3-PBX1-positive acute lymphoblastic leukemia in Austrian children

  • Leo Kager,
  • Thomas Lion,
  • Andishe Attarbaschi,
  • Margit Koenig,
  • Sabine Strehl,
  • Oskar A. Haas,
  • Michael N. Dworzak,
  • Martin Schrappe,
  • Helmut Gadner,
  • Georg Mann

DOI
https://doi.org/10.3324/haematol.11239
Journal volume & issue
Vol. 92, no. 11

Abstract

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Lessons from the analysis of children with TCF3-PBX1 ALL could help to identify treatment components essential for this leukemia subtype. Of 859 children with ALL who were treated in ALL-BFM trials in Austria, 31 (3.6%) had a TCF3-PBX1 ALL. The 5-year event-free survival rate for these 31 patients was 90%±5%. Patients with TCF3-PBX1 ALL treated on the ALL-BFM 86 trial had a poorer outcome than patients with TCF3-PBX1 ALL treated on later trials. These data document that contemporary ALL-BFM treatment is highly effective in children with TCF3-PBX1 ALL. Implementation of early dose-intensified remission induction may be an essential treatment component.