Haematologica, Vol 92, Issue 11, 1561-1564 doi:10.3324/haematol.11239
Copyright © 2007 by Ferrata Storti Foundation
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Acute Lymphoblastic Leukemia

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 for the Austrian BFM Study Group

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.




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