title: Acute lymphoblastic leukemia and Down syndrome: the collaborative study of the Tokyo Children's Cancer Study Group and the Kyushu Yamaguchi Children's Cancer Study Group.
abstract: The Tokyo Children's Cancer Study Group (TCCSG) and the Kyushu Yamaguchi Children's Cancer Study Group (KYCCSG) performed a collaborative analysis of data on children with Down syndrome and acute lymphoblastic leukemia (DS-ALL). Among the 1,139 patients who were enrolled in the TCCSG L99-15, L99-1502, or the KYCCSG ALL 96 study, 13 patients with newly diagnosed ALL had DS. In the DS patients, a significantly higher proportion of patients developed ALL at age 5 years or older compared with the non-DS ALL patients (P < 0.001). The 5-year relapse-free or overall survival of DS-ALL patients was 50.0 or 61.5%, respectively. Relapse accounted for all causes of death. In the TCCSG L99-15 cohort, the overall survival of DS-ALL patients was 42.9%, which was significantly worse compared with 87.9% in the non-DS population (P < 0.001). The survival of patients who received reduced-dose chemotherapy was significantly worse than those who received full-dose chemotherapy (P < 0.001). However, a higher dose of methotrexate was not associated with a better outcome. Results of our preliminary study suggest that the survival of DS-ALL patients could be improved by treatment without dose reduction if possible, although the appropriate dose of methotrexate for DS-ALL needs to be determined.
authors.affiliation: Department of Pediatrics, Yokohama City University, School of Medicine, 3-9 Fukuura Kanazawa-ku, Yokohama, 236-0004, Japan. hgoto39@med.yokohama-cu.ac.jp.
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Goto
authors.affiliation: Department of Pediatrics, University of Yamanashi, School of Medicine, Kohu, Japan.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Inukai
authors.affiliation: Division of Oncology, Kanagawa Children's Medical Center, Yokohama, Japan.
authors.forename: Hiroyasu
authors.initials: H
authors.lastname: Inoue
authors.affiliation: Department of Pediatrics, St. Luke's International Hospital, Tokyo, Japan.
authors.forename: Chitose
authors.initials: C
authors.lastname: Ogawa
authors.affiliation: Department of Pediatrics, School of Medicine, University of Tsukuba, Tsukuba, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Fukushima
authors.affiliation: Department of Cell Transplantation, Tokai University Hospital, Isehara, Japan.
authors.forename: Miharu
authors.initials: M
authors.lastname: Yabe
authors.affiliation: Department of Pediatrics, Faculty of Medicine, University of Tokyo, Tokyo, Japan.
authors.forename: Akira
authors.initials: A
authors.lastname: Kikuchi
authors.affiliation: Department of Pediatric Hematology and Oncology, Ibaraki Children's Hospital, Mito, Japan.
authors.forename: Kazutoshi
authors.initials: K
authors.lastname: Koike
authors.affiliation: Department of Pediatrics, Dokkyo Medical University, Mibu, Japan.
authors.forename: Keitaro
authors.initials: K
authors.lastname: Fukushima
authors.affiliation: Department of Pediatrics, Showa University, School of Medicine, Fujigaoka Hospital, Yokohama, Japan.
authors.forename: Keiichi
authors.initials: K
authors.lastname: Isoyama
authors.affiliation: Department of Health Policy, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Tomohiro
authors.initials: T
authors.lastname: Saito
authors.affiliation: Department of First Pediatrics, Toho University Medical Center, Oomori Hospital, Tokyo, Japan.
authors.forename: Akira
authors.initials: A
authors.lastname: Ohara
authors.affiliation: Department of Hematology/Oncology, Saitama Children's Medical Center, Iwatsuki, Japan.
authors.forename: Ryoji
authors.initials: R
authors.lastname: Hanada
authors.affiliation: Department of Pediatrics, Aso-Iizuka Hospital, Iizuka, Japan.
authors.forename: Jiro
authors.initials: J
authors.lastname: Iwamoto
authors.affiliation: Department of Pediatrics, Yamaguchi University Graduate School of Medicine, Yamaguchi, Japan.
authors.forename: Noriko
authors.initials: N
authors.lastname: Hotta
authors.affiliation: Section of Pediatrics, National Kyushu Cancer Centre, Fukuoka, Japan.
authors.forename: Yoshihisa
authors.initials: Y
authors.lastname: Nagatoshi
authors.affiliation: Section of Pediatrics, National Kyushu Cancer Centre, Fukuoka, Japan.
authors.forename: Jun
authors.initials: J
authors.lastname: Okamura
authors.affiliation: Department of Pediatric Hematology and Oncology, Ibaraki Children's Hospital, Mito, Japan.
authors.forename: Masahiro
authors.initials: M
authors.lastname: Tsuchida
mesh_terms: D000293:Adolescent; D000970:Antineoplastic Agents / Q000008:administration & dosage* / Q000627:therapeutic use; D002648:Child; D015331:Cohort Studies; D004314:Down Syndrome / Q000150:complications* / Q000188:drug therapy*; D005260:Female; D006801:Humans; D007564:Japan; D008297:Male; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000150:complications* / Q000188:drug therapy*; D016019:Survival Analysis
chemical_list: D000970:Antineoplastic Agents