| Literature DB >> 29589281 |
Hiroyuki Takahashi1, Ryosuke Kajiwara2, Motohiro Kato3, Daisuke Hasegawa4, Daisuke Tomizawa3, Yasushi Noguchi5, Kazutoshi Koike6, Daisuke Toyama7, Hiromasa Yabe8, Michiko Kajiwara9, Junya Fujimura10, Manabu Sotomatsu11, Setsuo Ota12, Miho Maeda13, Hiroaki Goto14, Yoko Kato15, Tetsuya Mori16, Takeshi Inukai17, Hiroyuki Shimada18, Keitaro Fukushima19, Chitose Ogawa20, Atsushi Makimoto21, Takashi Fukushima22, Kentaro Ohki23, Katsuyoshi Koh24, Nobutaka Kiyokawa23, Atsushi Manabe4, Akira Ohara25.
Abstract
The survival rate of children with acute lymphoblastic leukemia (ALL) has increased to approximately 90% after substantial progress in risk-oriented treatment strategies. Between 2005 and 2013, the Tokyo Children's Cancer Study Group (TCCSG) conducted a risk-oriented, non-randomized study, L04-16. The principal aim of this study was to assemble background characteristics and treatment outcomes, and gather genetic information on leukemic cells under central diagnosis. This report outlines the background characteristics and treatment outcomes of 1033 children with ALL treated according to a TCCSG platform. The 5-year event-free and overall survival (OS) rates for all children were 78.1 ± 1.3 and 89.6 ± 1.0%, respectively. The OS rate was significantly higher in children with B-cell precursor (BCP)-ALL (91.9 ± 1.0%, n = 916) than in those with T-ALL (71.9 ± 4.3%, n = 117, p < 0.001). In univariate analysis for BCP-ALL, children aged 1-6 years (5y-OS: 94.2 ± 1.0%), with an initial white blood cell count of < 20,000/μL (94.0 ± 1.0%), high hyperdiploidy (95.4 ± 1.6%), ETV6-RUNX1 (97.4 ± 1.2%) or TCF3-PBX1 (96.9 ± 2.1%), and "Day8NoBlasts" (96.4 ± 1.1%) had the best outcomes. Genetic investigation revealed two novel fusion genes within this cohort: ETV6-ZNF385A and ZNF362-TCF4. Our study highlighted the clinical aspects of genomic features of ALL in Japanese children. We provide fundamental information for the further molecular investigation of this disease.Entities:
Keywords: Acute lymphoblastic leukemia; Childhood leukemia; Clinical trial; Novel fusion genes
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Year: 2018 PMID: 29589281 DOI: 10.1007/s12185-018-2440-4
Source DB: PubMed Journal: Int J Hematol ISSN: 0925-5710 Impact factor: 2.490