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

From the St. Anna Children’s Hospital (LK, AA, HG, OAH, MND, GM); Children’s Cancer Research Institute, Vienna, Austria (TL, MK, SS, HG); Department of Pediatrics, University Hospital Schleswig-Holstein, Campus Kiel, Kiel, Germany (MS)

Correspondence: Georg Mann, MD, Department of Hematology and Oncology, St. Anna Children’s Hospital, Kinderspitalgasse 6, A-1090 Vienna, Austria. E-mail: georg.mann{at}stanna.at

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.

Key words: outcome, TCF3-PBX1, acute lymphoblastic leukemia.