|Title:||Pediatric acute lymphoblastic leukemia with t(1;19)/TCF3-PBX1 in Taiwan||Authors:||Yen H.-J.
|Issue Date:||2017||Publisher:||John Wiley and Sons Inc.||Journal Volume:||64||Journal Issue:||10||Start page/Pages:||e26557||Source:||Pediatric Blood and Cancer||Abstract:||
Background: In childhood acute lymphoblastic leukemia (ALL), t(1;19)(q23;p13.3) with TCF3-PBX1 fusion is one of the most frequent translocations. Historically, it has been associated with poor prognosis. Intensive treatment, however, has improved its outcome. We determined the outcome of children with this genotype treated with contemporary intensive chemotherapy in Taiwan. Procedure: In Taiwan Pediatric Oncology Group 2002 ALL studies, genotypes were determined by cytogenetic analysis and/or reverse transcriptase polymerase chain reaction assay. Based on presenting features, immunophenotype and genotype, patients were assigned to one of the three risk groups: standard risk (SR), high risk (HR), or very high risk (VHR). The patients with t(1;19)/TCF3-PBX1 were treated in the HR arm receiving more intensive chemotherapy. The outcomes of patients with t(1;19)/TCF3-PBX1 were compared to that of patients with other subtypes of B-precursor ALL (B-ALL). Results: Of the 1,129 patients with B-ALL, 64 (5.7%) had t(1;19)/TCF3-PBX1; 51 of whom were treated in the HR arm, but 11 were treated in the VHR and 2 in the SR arm because of physician's preference. As a group, 64 patients with t(1;19)/TCF3-PBX1 had similar 5-year event-free survival (83.3 ± 4.8%) as those with TEL-AML1 (85.2 ± 3.4%, P = 0.984) or those with hyperdiploidy >50 (84.0 ± 3.1%, P = 0.748). The cumulative risk of any (isolated plus combined) central nervous system relapse among patients with t(1;19)/TCF3-PBX1 (8.7 ± 3.8%) tended to be higher than that of patients with TEL-AML1 (5.8 ± 2.3%, P = 0.749) or those with hyperdiploidy (4.1 ± 1.8%, P = 0.135), albeit the differences did not reach statistical significance. Conclusions: With contemporary intensive chemotherapy, children with t(1;19)/TCF3-PBX1 fared as well as those with favorable genotypes (TEL-AML1 or hyperdiploidy). ? 2017 Wiley Periodicals, Inc.
|ISSN:||1545-5009||DOI:||10.1002/pbc.26557||SDG/Keyword:||asparaginase; dexamethasone; epirubicin; mercaptopurine; methotrexate; prednisone; vincristine; oncoprotein; TCF3-PBX1 fusion protein, human; acute lymphoblastic leukemia; adolescent; adult; Article; cancer chemotherapy; cancer recurrence; cancer survival; central nervous system; child; childhood leukemia; chromosome analysis; chromosome translocation; consolidation chemotherapy; diploidy; drug megadose; event free survival; female; fusion gene; genotype; high risk patient; human; immunophenotyping; induction chemotherapy; infant; leukemia relapse; leukemia remission; major clinical study; male; multiple cycle treatment; overall survival; priority journal; reverse transcription polymerase chain reaction; Taiwan; TCF3 PBX1 fusion gene; TEL AML1 fusion gene; treatment outcome; chromosome 1; chromosome 19; clinical trial; gene translocation; genetics; metabolism; multicenter study; newborn; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma; preschool child; Taiwan; Adolescent; Child; Child, Preschool; Chromosomes, Human, Pair 1; Chromosomes, Human, Pair 19; Female; Humans; Infant; Infant, Newborn; Male; Oncogene Proteins, Fusion; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma; Taiwan; Translocation, Genetic
|Appears in Collections:||醫學系|
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