title: An overall characterization of pediatric acute lymphoblastic leukemia with CRLF2 overexpression.
abstract: For an overall characterization of pediatric B-cell precursor acute lymphoblastic leukemia (BCPALL) with CRLF2 overexpression (OE), we conducted genetic analysis of CRLF2 in 167 pediatric BCPALL patients. CRLF2 OE was detected in 30 (18%) of 167 patients, the P2RY8-CRLF2 fusion was identified in only 3 (1.8%) of 167 patients, all of which demonstrated CRLF2 OE. Moreover, CRLF2 gain was identified in 18 (11%) of 167 patients. Messenger RNA sequencing revealed a novel fusion transcript, CSF2RA-CRLF2, in a case with CRLF2 OE, suggesting that this fusion is associated with CRLF2 OE. In survival analysis, no significant differences in 5-year event-free survival (EFS) and overall survival were observed between patients with and without CRLF2 OE (70.7 vs. 75.4%, log rank P = 0.68 and 96.4 vs. 82.1%, log rank P = 0.11, respectively). However, a significant difference in 5-year EFS between CRLF2 OE patients with and without IKZF1 deletion was observed (44.4 vs. 83.1%, log rank P = 0.02). In multivariate analysis, only IKZF1 deletion was a significant predictor of inferior OS (hazard ratio: 2.427, P = 0.04).These findings suggest that CRLF2 OE is not an independent prognostic factor in pediatric BCPALL.
authors.affiliation: Department of Pediatrics, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, Kyoto, Japan.
authors.forename: Mio
authors.initials: M
authors.lastname: Yano
authors.forename: Toshihiko
authors.initials: T
authors.lastname: Imamura
authors.forename: Daisuke
authors.initials: D
authors.lastname: Asai
authors.forename: Akiko
authors.initials: A
authors.lastname: Moriya-Saito
authors.forename: So-ichi
authors.initials: S
authors.lastname: Suenobu
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: Hirohide
authors.initials: H
authors.lastname: Kawasaki
authors.forename: Hiroki
authors.initials: H
authors.lastname: Hori
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Kosaka
authors.forename: Koji
authors.initials: K
authors.lastname: Kato
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: Kenji
authors.initials: K
authors.lastname: Matsumoto
authors.forename: Nobutaka
authors.initials: N
authors.lastname: Kiyokawa
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; D005260:Female; D050939:Gene Fusion; D006801:Humans; D051740:Ikaros Transcription Factor / Q000235:genetics / Q000378:metabolism; D007223:Infant; D008297:Male; D010372:Pediatrics; D015452:Precursor B-Cell Lymphoblastic Leukemia-Lymphoma / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology; D011379:Prognosis; D018121:Receptors, Cytokine / Q000235:genetics* / Q000378:metabolism; D016187:Receptors, Granulocyte-Macrophage Colony-Stimulating Factor / Q000235:genetics / Q000378:metabolism; D058470:Receptors, Purinergic P2Y / Q000235:genetics / Q000378:metabolism; D016019:Survival Analysis
chemical_list: C498762:CRLF2 protein, human; C521678:CSF2RA protein, human; C497239:IKZF1 protein, human; C578279:P2RY8 protein, human; D018121:Receptors, Cytokine; D016187:Receptors, Granulocyte-Macrophage Colony-Stimulating Factor; D058470:Receptors, Purinergic P2Y; D051740:Ikaros Transcription Factor