Abstract
Although deletions of cell cycle regulatory gene loci have long been reported in various malignancies, little is known regarding their relevance in pediatric T-cell lymphoblastic lymphoma (T-LBL) and T-cell lymphoblastic leukemia (TALL). The current study focused on loss of heterozygosity (LOH) analyses of the CDKN2A/B (chromosome 9p), ATM (chromosome 11q) and p53 (chromosome 17p) gene loci. Frequencies of LOH were compared in 113 pediatric T-LBL and 125 T-ALL who were treated uniformly according to ALL-BFM strategies. Furthermore, LOH findings were correlated with clinical characteristics and tested for their prognostic relevance. LOH at 9p was detected in 47% of T-LBL and 51% of T-ALL, and was associated with male gender in both. In T-ALL, LOH at 9p was associated with favorable initial treatment response. A tendency for favorable event-free-survival was observed in LOH 9p positive T-LBL. The frequency of LOH at chromosomes 11q and 17p was 5% or less for both diseases.
MeSH terms
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Adolescent
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Ataxia Telangiectasia Mutated Proteins
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Cell Cycle Proteins / genetics*
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Child
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Cyclin-Dependent Kinase Inhibitor p15 / genetics*
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Cyclin-Dependent Kinase Inhibitor p16 / genetics*
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DNA, Neoplasm / genetics
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DNA-Binding Proteins / genetics*
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Female
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Genetic Loci / genetics*
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Genetic Markers / genetics
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Humans
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Leukemia, T-Cell / genetics*
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Leukemia, T-Cell / mortality
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Leukemia, T-Cell / pathology
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Loss of Heterozygosity / genetics
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Male
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Microsatellite Repeats / genetics*
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Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / diagnosis
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Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / genetics*
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Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / mortality
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Protein Serine-Threonine Kinases / genetics*
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Survival Rate / trends
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Tumor Suppressor Protein p53 / genetics*
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Tumor Suppressor Proteins / genetics*
Substances
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CDKN2B protein, human
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Cell Cycle Proteins
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Cyclin-Dependent Kinase Inhibitor p15
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Cyclin-Dependent Kinase Inhibitor p16
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DNA, Neoplasm
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DNA-Binding Proteins
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Genetic Markers
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Tumor Suppressor Protein p53
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Tumor Suppressor Proteins
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ATM protein, human
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Ataxia Telangiectasia Mutated Proteins
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Protein Serine-Threonine Kinases