Abstract
To evaluate the impact of contemporary therapy on the clinical outcome of children with pre-B acute lymphoblastic leukemia (ALL) and the t(1;19)/TCF3/PBX1, we analyzed 735 patients with B-cell precursor ALL treated in four successive protocols at St Jude Children's Research Hospital. The 41 patients with the t(1;19) had a comparable event-free survival to that of the 694 patients with other B-cell precursor ALL (P=0.63; 84.2+/-7.1% (s.e.) vs 84.0+/-1.8% at 5 years). However, patients with the t(1;19) had a lower cumulative incidence of any hematological relapse (P=0.06; 0 vs 8.3+/-1.2% at 5 years) but a significantly higher incidence of central nervous system (CNS) relapse (P<0.001; 9.0+/-5.1% vs 1.0+/-0.4% at 5 years). In a multivariate analysis, the t(1;19) was an independent risk factor for isolated CNS relapse. These data suggest that with contemporary treatment, patients with the t(1;19) and TCF3/PBX1 fusion have a favorable overall outcome but increased risk of CNS relapse.
Publication types
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Research Support, N.I.H., Extramural
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Research Support, Non-U.S. Gov't
MeSH terms
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Adolescent
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Antineoplastic Combined Chemotherapy Protocols / administration & dosage
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Antineoplastic Combined Chemotherapy Protocols / therapeutic use
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Central Nervous System / pathology*
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Child
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Child, Preschool
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Chromosomes, Human, Pair 1 / genetics
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Chromosomes, Human, Pair 1 / ultrastructure*
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Chromosomes, Human, Pair 19 / genetics
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Chromosomes, Human, Pair 19 / ultrastructure*
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Clinical Trials as Topic / statistics & numerical data
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Combined Modality Therapy
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Cranial Irradiation
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Disease-Free Survival
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Female
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Genotype
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Humans
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Incidence
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Infant
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Injections, Spinal
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Leukemic Infiltration / epidemiology*
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Leukemic Infiltration / prevention & control
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Male
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Oncogene Proteins, Fusion / genetics*
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Precursor B-Cell Lymphoblastic Leukemia-Lymphoma / drug therapy
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Precursor B-Cell Lymphoblastic Leukemia-Lymphoma / epidemiology
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Precursor B-Cell Lymphoblastic Leukemia-Lymphoma / genetics*
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Precursor B-Cell Lymphoblastic Leukemia-Lymphoma / pathology
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Precursor B-Cell Lymphoblastic Leukemia-Lymphoma / radiotherapy
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Prognosis
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Proportional Hazards Models
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Risk
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Risk Assessment
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Translocation, Genetic*
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Treatment Outcome
Substances
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Oncogene Proteins, Fusion
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TCF3-PBX1 fusion protein, human