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Nationwide survey of pediatric hypodiploid acute lymphoblastic leukemia in Japan
Author(s) -
Ishimaru Sae,
Okamoto Yasuhiro,
Imai Chihaya,
Sakaguchi Hirotoshi,
Taki Tomohiko,
Hasegawa Daisuke,
Cho Yuko,
Kakuda Harumi,
Sano Hideki,
Manabe Atsushi,
Imamura Toshihiko,
Kato Motohiro,
Arakawa Yuki,
Shimonodan Hidemi,
Sato Atsushi,
Suenobu Souichi,
Inukai Takeshi,
Watanabe Arata,
Kawano Yoshifumi,
Kikuta Atsushi,
Horibe Keizo,
Ohara Akira,
Koh Katsuyoshi
Publication year - 2019
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/ped.14006
Subject(s) - medicine , lymphoblastic leukemia , prednisolone , hematopoietic stem cell transplantation , chemotherapy , transplantation , pediatrics , prospective cohort study , multicenter study , clinical trial , leukemia , randomized controlled trial
Background Ploidy is a highly significant prognostic factor for pediatric acute lymphoblastic leukemia ( ALL ). Children with hypodiploid ALL have poor outcomes despite current intensive chemotherapy. Little has been investigated with regard to hypodiploid ALL in Japanese children. Methods We retrospectively collected clinical data on hypodiploid ALL cases from the registries of prospective multicenter trials conducted by the four independent clinical study groups in Japan between 1997 and 2012. Results A total of 117 ALL patients with hypodiploidy were analyzed in this study. There were 101, eight, and eight patients with 45, 44, and fewer than 44 chromosomes, respectively. The 5 year overall survival rates differed significantly: 86.0%, 87.5%, and 62.5% for patients with 45, 44, and fewer than 44 chromosomes, respectively ( P = 0.037). Of the eight patients with 44 chromosomes, seven were alive, including five patients who maintained complete remission without undergoing hematopoietic stem cell transplantation ( HSCT ). Of the eight patients with fewer than 44 chromosomes, six were good responders to prednisolone and none had induction failure, but the relapse rate was high (5/8). No patients had central nervous system relapse. Four patients underwent HSCT after relapse, but only one survived. Conclusions Outcomes of Japanese ALL patients with fewer than 44 chromosomes were poor, as previously reported in other countries. Although the sample size was small, patients with 44 chromosomes had better prognoses than those previously reported. Further studies including international collaboration are needed to improve outcomes for pediatric ALL patients with fewer than 44 chromosomes.

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