Incidence and outcome of TCF3-PBX1-positive acute lymphoblastic leukemia in Austrian children
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Published on: May 10, 2017
Leo Kager1, Thomas Lion, Andishe Attarbaschi
1Department of Hematology and Oncology, St. Anna Children's Hospital, Kinderspitalgasse 6, A-1090 Vienna, Austria.
Insights
TCF3-PBX1 Acute Lymphoblastic Leukemia (ALL) in children is effectively treated with contemporary ALL-BFM protocols. Early dose-intensified remission induction appears crucial for successful outcomes in this leukemia subtype.
Area of Science:
- Pediatric Oncology
- Hematology
- Leukemia Research
Background:
- TCF3-PBX1 fusion gene is a specific subtype of childhood Acute Lymphoblastic Leukemia (ALL).
- Understanding treatment efficacy for rare leukemia subtypes is critical for improving patient outcomes.
Purpose of the Study:
- To analyze the outcomes of children with TCF3-PBX1 ALL treated within the ALL-BFM trials.
- To identify essential treatment components for this specific leukemia subtype.
Main Methods:
- Retrospective analysis of 859 children with ALL treated in Austrian ALL-BFM trials.
- Identification of patients with the TCF3-PBX1 genetic alteration.
- Evaluation of 5-year event-free survival rates.
Main Results:
- 3.6% of children (31 patients) were diagnosed with TCF3-PBX1 ALL.
- The 5-year event-free survival rate for this cohort was 90%+/-5%.
- Patients treated on earlier ALL-BFM 86 trial showed poorer outcomes compared to those on later trials.
Conclusions:
- Contemporary ALL-BFM treatment demonstrates high effectiveness in children with TCF3-PBX1 ALL.
- Early dose-intensified remission induction is likely a key component for successful treatment of TCF3-PBX1 ALL.
Abstract:
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.
