TY - JOUR
T1 - Treatment outcome of children with acute lymphoblastic leukemia
T2 - the Tokyo Children’s Cancer Study Group (TCCSG) Study L04-16
AU - Takahashi, Hiroyuki
AU - Kajiwara, Ryosuke
AU - Kato, Motohiro
AU - Hasegawa, Daisuke
AU - Tomizawa, Daisuke
AU - Noguchi, Yasushi
AU - Koike, Kazutoshi
AU - Toyama, Daisuke
AU - Yabe, Hiromasa
AU - Kajiwara, Michiko
AU - Fujimura, Junya
AU - Sotomatsu, Manabu
AU - Ota, Setsuo
AU - Maeda, Miho
AU - Goto, Hiroaki
AU - Kato, Yoko
AU - Mori, Tetsuya
AU - Inukai, Takeshi
AU - Shimada, Hiroyuki
AU - Fukushima, Keitaro
AU - Ogawa, Chitose
AU - Makimoto, Atsushi
AU - Fukushima, Takashi
AU - Ohki, Kentaro
AU - Koh, Katsuyoshi
AU - Kiyokawa, Nobutaka
AU - Manabe, Atsushi
AU - Ohara, Akira
N1 - Funding Information:
Acknowledgements The authors would like to thank the participating institutes and physicians in the TCCSG. They would also like to thank Ms. Kaori Itagaki for preparing and refining the protocol data. This work was supported, in part, by grants from the Children’s Cancer Association of Japan and the Grant of the National Center for Child Health and Development (26-20).
Publisher Copyright:
© 2018, The Japanese Society of Hematology.
PY - 2018/7/1
Y1 - 2018/7/1
N2 - 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.
AB - 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.
KW - Acute lymphoblastic leukemia
KW - Childhood leukemia
KW - Clinical trial
KW - Novel fusion genes
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UR - http://www.scopus.com/inward/citedby.url?scp=85044472364&partnerID=8YFLogxK
U2 - 10.1007/s12185-018-2440-4
DO - 10.1007/s12185-018-2440-4
M3 - Article
C2 - 29589281
AN - SCOPUS:85044472364
SN - 0925-5710
VL - 108
SP - 98
EP - 108
JO - International Journal of Hematology
JF - International Journal of Hematology
IS - 1
ER -
