International Journal of Hematology

DOI: 10.1007/s12185-019-02599-w Pages: 477-482

Discontinuation of l-asparaginase and poor response to prednisolone are associated with poor outcome of ETV6-RUNX1-positive pediatric B-cell precursor acute lymphoblastic leukemia

1. Hyogo Prefectural Amagasaki General Medical Center, Department of Pediatric Hematology and Oncology

2. National Hospital Organization Nagoya Medical Center, Clinical Research Center

3. Kyoto Prefectural University of Medicine, Department of Pediatrics, Graduate School of Medical Science

4. Aomori Prefectural Central Hospital, Department of Pediatrics

5. Oita University, Division of General Pediatrics and Emergency Medicine, Department of Pediatrics

6. Hyogo Prefectural Children’s Hospital, Department of Hematology/Oncology

7. Osaka University, Department of Pediatrics

8. Mie University, Department of Pediatrics

9. Sapporo Medical University of Medicine, Department of Pediatrics

10. Okayama University, Department of Pediatrics

11. Japanese Red Cross Nagoya First Hospital, Department of Hematology Oncology, Children’s Medical Center

12. Hirosaki University, Department of Pediatrics

13. Kansai Medical University, Department of Pediatrics

14. Yumura Clinic

15. Osaka City General Hospital, Department of Pediatric Hematology/Oncology

16. Miyagi Children’s Hospital, Department of Hematology/Oncology

Correspondence to:
Toshihiko Imamura
Tel: 81-75-251-5571



ETV6-RUNX1-positive B precursor acute lymphoblastic leukemia (B-ALL) is a common subtype of pediatric B-ALL that has shown excellent outcomes in contemporary clinical trials for pediatric B-ALL. Examinations of the possibility of reducing therapeutic intensity may thus be explored. This prospective study examined outcomes in 205 pediatric patients with ETV6-RUNX1-positive B-ALL uniformly treated following the Japan Association of Childhood Leukemia Study Group (JACLS) ALL-02 protocol. The JACLS ALL-02 protocol does not employ minimal residual disease detected by polymerase chain reaction (PCR-MRD)-based risk stratification; however, 4-year event-free survival (EFS) and overall survival (OS) were 94.4 ± 1.6 and 97.5 ± 1.1%, respectively. In particular, 92 of 205 (44.9%) patients were successfully treated with a less intensive regimen involving only two cycles of high dose methotrexate and one course of re-induction therapy comprising vincristine, l-asparaginase (L-asp), pirarubicin, and prednisolone. Multivariate analysis revealed that discontinuation of L-asp and poor response to prednisolone was, respectively, associated with poor EFS (HR 6.3; 95% CI 1.3–27.0) and OS (HR 17.5; 95% CI 2.3–130), suggesting that the majority of ETV6-RUNX1-positive B-ALL cases may be cured by a less-intensive chemotherapy regimen if the risk stratification system including PCR-MRD monitoring and insufficient use of L-asp is avoided.

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