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Sakaguchi, Kimiyoshi Department of Pediatrics, Hamamatsu University School of Medicine, Hamamatsu, Japan Clinical Research Center, National Hospital Organization Nagoya Medical Center, Nagoya, Japan
Imamura, Toshihiko Clinical Research Center, National Hospital Organization Nagoya Medical Center, Nagoya, Japan Department of Pediatrics, Kyoto Prefectural University of Medicine, Kyoto, Japan
Ishimaru, Sae Department of Hematology and Oncology, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan Department of Pediatric Oncology, National Cancer Center Hospital, Tokyo, Japan
Imai,, Chihaya Department of Pediatrics, Niigata University, Niigata, Japan
Shimonodan, Hidemi Department of Pediatrics, Miyazaki Prefectural Miyazaki Hospital, Miyazaki, Japan
Fujita, Naoto Department of Pediatrics, Hiroshima Red Cross Hospital and Atomic-bomb Survivors Hospital, Hiroshima, Japan
Okada, Keiko Department of Pediatric Hematology/Oncology, Osaka City General Hospital, Osaka, Japan
Tauchi, Hisamichi Department of Pediatrics, Ehime University, Toon, Japan
Kato, Motohiro Division of Stem Cell Transplant and Cellular Therapy, Children's Cancer Center, National Center for Child Health and Development, Tokyo, Japan
Kojima, Yasuko Department of Pediatrics, Toho University Omori Medical Center, Tokyo, Japan
Watanabe, Arata Department of Pediatrics, Nakadori General Hospital, Akita, Japan
Deguchi, Takao Department of Pediatrics, Mie University, Tsu, Japan
Hashii, Yoshiko Department of Pediatrics, Osaka University, Suita, Japan
Kiyokawa, Nobutaka Department of Pediatric Hematology and Oncology Research, Research Institute, National Center for Child Health and Development, Tokyo, Japan
Taki, Tomohiko Department of Medical Technology, Kyorin University Faculty of Health Sciences, Mitaka, Japan
Saito Akiko M. Clinical Research Center, National Hospital Organization Nagoya Medical Center, Nagoya, Japan
Horibe, Keizo Clinical Research Center, National Hospital Organization Nagoya Medical Center, Nagoya, Japan
Manabe, Atsushi Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Japan
Sato, Atsushi Department of Hematology/Oncology, Miyagi Children's Hospital, Sendai, Japan
Koh, Katsuyoshi Department of Hematology/Oncology, Saitama Children's Medical Center, Saitama, Japan
Description
Background
Rearrangements of chromosome 8q24/MYC (8q24/MYC-r), resulting from t(8;14)(q24;q32), t(2;8)(p11;q24), or t(8;22)(q24;q11), are mainly associated with Burkitt lymphoma/leukemia (BL) and rarely observed in patients with B-cell precursor acute lymphoblastic leukemia (BCP-ALL). The characteristics of BCP-ALL with 8q24/MYC-r are poorly understood.

Procedure
A retrospective nationwide study of data from patients with pediatric BCP-ALL with 8q24/MYC-r in Japan was conducted to clarify the clinical and biological characteristics associated with 8q24/MYC-r BCP-ALL.

Results
Ten patients with BCP-ALL with 8q24/MYC-r, including three with double-hit leukemia (DHL) (two with t(8;14)(q24;q32) and t(14;18)(q32;q21) and one with t(8;14) and t(3;22)(q27;q11)), were identified. Patients with BCP-ALL with 8q24/MYC-r had higher median age and uric acid and lactate dehydrogenase levels, than those without 8q24/MYC-r. All patients were initially treated with ALL-type chemotherapy; however, four, including one with DHL, were switched to BL-type chemotherapy, based on cytogenetic findings. One patient relapsed after standard-risk ALL-type chemotherapy, and two patients with DHL did not attain complete remission with chemotherapy; all three died within 11 months. The other seven patients treated with BL-type or high-risk ALL-type chemotherapy are alive without disease.

Conclusions
The clinical and laboratory features of BL with IG-MYC rearrangement, displaying a BCP immunophenotype (Wagener et al. and Herbrueggen et al. termed it as pre-BLL), are similar to those of BCP-ALL with 8q24/MYC-r. Low-risk ALL-type chemotherapy may not be appropriate for them, and further studies are required to establish an adequate therapeutic strategy. Further studies of DHL to identify new treatment strategies are also needed.
Journal Title
Pediatric blood & cancer
Volume
67
Issue
7
ISSN
1545-5009
ISSN(Online)
1545-5017
Published Date
2020-04-23
DOI
PubMed ID
Publisher
Hoboken, N.J. : John Wiley, c 2004-
NII Type
Journal Article
Format
PDF
Text Version
著者版
Gyoseki ID
39958
OAI-PMH Set
Faculty of Medicine
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