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.
MeSH terms
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Adolescent
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Antineoplastic Combined Chemotherapy Protocols / therapeutic use
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Asparaginase / therapeutic use
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Austria / epidemiology
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Basic Helix-Loop-Helix Transcription Factors / genetics
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Child
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Child, Preschool
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DNA-Binding Proteins / genetics
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Daunorubicin / therapeutic use
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Humans
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Incidence
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Infant
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Pre-B-Cell Leukemia Transcription Factor 1
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Precursor Cell Lymphoblastic Leukemia-Lymphoma / drug therapy*
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Precursor Cell Lymphoblastic Leukemia-Lymphoma / epidemiology*
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Precursor Cell Lymphoblastic Leukemia-Lymphoma / mortality
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Prednisone / therapeutic use
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Proto-Oncogene Proteins / genetics
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Survival Rate
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Treatment Outcome
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Vincristine / therapeutic use
Substances
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Basic Helix-Loop-Helix Transcription Factors
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DNA-Binding Proteins
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Pre-B-Cell Leukemia Transcription Factor 1
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Proto-Oncogene Proteins
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TCF3 protein, human
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PBX1 protein, human
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Vincristine
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Asparaginase
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Prednisone
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Daunorubicin