title: Outcome of TCF3-PBX1 positive pediatric acute lymphoblastic leukemia patients in Japan: a collaborative study of Japan Association of Childhood Leukemia Study (JACLS) and Children's Cancer and Leukemia Study Group (CCLSG).
abstract: This study reviewed the clinical characteristics of 112 pediatric B-cell precursor acute lymphoblastic leukemia (BCP-ALL) patients with TCF3-PBX1 fusion treated according to the Japan Association of Childhood Leukemia Study (JACLS) ALL02 protocol (n = 82) and Children's Cancer and Leukemia Study Group (CCLSG) ALL 2004 protocol (n = 30). The 3-year event-free survival (EFS) and overall survival (OS) rates were 85.4 ± 3.9% and 89.0 ± 3.5% in JACLS cohort, and the 5-year EFS and OS were 82.8 ± 7.0% and 86.3 ± 6.4% in CCLSG cohort, respectively, which are comparable to those reported in western countries. Conventional prognostic factors such as age at onset, initial white blood cell count, and National Cancer Institute risk have also no impact on OS in both cohorts. Surprisingly, the pattern of relapse in JACLS cohort, 9 of 82 patients, was unique: eight of nine patients relapsed during the maintenance phase and one patient had primary induction failure. However, bone marrow status and assessment of minimal residual disease on days 15 and 33 did not identify those patients. Interestingly, the two patients with IKZF1 deletion eventually relapsed in JACLS cohort, as did one patient in CCLSG cohort. International collaborative study of larger cohort is warranted to clarify the impact of the IKZF1 deletion on the poor outcome of TCF3-PBX1 positive BCP-ALL.
authors.affiliation: Department of Pediatrics, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, Kyoto, Japan.
authors.forename: Daisuke
authors.initials: D
authors.lastname: Asai
authors.forename: Toshihiko
authors.initials: T
authors.lastname: Imamura
authors.forename: Yuka
authors.initials: Y
authors.lastname: Yamashita
authors.forename: So-ichi
authors.initials: S
authors.lastname: Suenobu
authors.forename: Akiko
authors.initials: A
authors.lastname: Moriya-Saito
authors.forename: Daiichiro
authors.initials: D
authors.lastname: Hasegawa
authors.forename: Takao
authors.initials: T
authors.lastname: Deguchi
authors.forename: Yoshiko
authors.initials: Y
authors.lastname: Hashii
authors.forename: Mikiya
authors.initials: M
authors.lastname: Endo
authors.forename: Naoki
authors.initials: N
authors.lastname: Hatakeyama
authors.forename: Hirohide
authors.initials: H
authors.lastname: Kawasaki
authors.forename: Hiroki
authors.initials: H
authors.lastname: Hori
authors.forename: Keizo
authors.initials: K
authors.lastname: Horibe
authors.forename: Keiko
authors.initials: K
authors.lastname: Yumura-Yagi
authors.forename: Junichi
authors.initials: J
authors.lastname: Hara
authors.forename: Arata
authors.initials: A
authors.lastname: Watanabe
authors.forename: Atsushi
authors.initials: A
authors.lastname: Kikuta
authors.forename: Megumi
authors.initials: M
authors.lastname: Oda
authors.forename: Atsushi
authors.initials: A
authors.lastname: Sato
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D004252:DNA Mutational Analysis; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D051740:Ikaros Transcription Factor / Q000235:genetics; D007223:Infant; D007564:Japan; D008297:Male; D015514:Oncogene Proteins, Fusion / Q000235:genetics*; D015452:Precursor B-Cell Lymphoblastic Leukemia-Lymphoma / Q000188:drug therapy* / Q000235:genetics* / Q000473:pathology; D011379:Prognosis; D017384:Sequence Deletion; D016159:Tumor Suppressor Protein p53 / Q000235:genetics
chemical_list: C497239:IKZF1 protein, human; D015514:Oncogene Proteins, Fusion; C543638:TCF3-PBX1 fusion protein, human; C495901:TP53 protein, human; D016159:Tumor Suppressor Protein p53; D051740:Ikaros Transcription Factor
keywords: IKZF1 deletion; TCF3-PBX1; pediatric acute lymphoblastic leukemia