International Journal of Hematology

DOI: 10.1007/s12185-018-2440-4 Pages: 98-108

Treatment outcome of children with acute lymphoblastic leukemia: the Tokyo Children’s Cancer Study Group (TCCSG) Study L04-16

1. Toho University, Department of Pediatrics

2. Yokohama City University, Department of Pediatrics

3. Children’s Cancer Center, National Center for Child Health and Development

4. St Luke’s International Hospital, Department of Pediatrics

5. Japanese Red Cross Narita Hospital, Department of Pediatrics

6. Ibaraki Children’s Hospital, Division of Pediatric Hematology and Oncology

7. Showa University Fujigaoka Hospital, Department of Pediatrics

8. Tokai University School of Medicine, Department of Cell Transplantation and Regenerative Medicine

9. Tokyo Medical and Dental University, Department of Pediatrics

10. Juntendo University, Department of Pediatrics

11. Gunma Children’s Medical Center, Department of Pediatrics

12. Teikyo University Chiba Medical Center, Department of Pediatrics

13. Nippon Medical School, Department of Pediatrics

14. Kanagawa Children’s Medical Center, Division of Hemato-Oncology/Regenerative Medicine

15. Jikei University School of Medicine, Department of Pediatrics

16. St. Marianna University, Department of Pediatrics

17. University of Yamanashi, Department of Pediatrics

18. Keio University School of Medicine, Department of Pediatrics

19. Dokkyo Medical University, Department of Pediatrics

20. National Cancer Center Hospital, Department of Pediatric Oncology

21. Tokyo Metropolitan Children’s Medical Center, Department of Hematology-Oncology

22. University of Tsukuba, Department of Pediatrics

23. National Research Institute for Child Health and Development, Department of Pediatric Hematology and Oncology Research

24. Saitama Children’s Medical Center, Department of Hematology/Oncology

Correspondence to:
Hiroyuki Takahashi
Tel: 81-3-3762-4151
Email: hiroyuki.takahashi@med.toho-u.ac.jp

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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.

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