title: Monosomy 7 and deletion 7q in children and adolescents with acute myeloid leukemia: an international retrospective study.
abstract: Monosomy 7 (-7) and deletion 7q \del(7q)] are rare in childhood acute myeloid leukemia (AML). We retrospectively collected data on 258 children with AML or refractory anemia with excess blasts in transformation (RAEB-T) and -7 or del(7q) with or without other cytogenetic aberrations \+/- other]. Karyotypes included -7 (n = 90), -7 other (n = 82), del(7q) (n = 21), and del(7q) other (n = 65). Complete remission (CR) was achieved in fewer patients with -7 +/- other compared with del(7q) +/- other (61% versus 89%, P < .001). Overall, the 5-year survival rate was 39% (SE, 3%). Survival was superior in del(7q) +/- other compared with -7 +/- other (51% versus 30%, P < .01). Cytogenetic aberrations considered favorable in AML \t(8;21)(q22;q22), inv(16)(p13q22), t(15;17)(q22;q21), t(9;11)(p22;q23)] (n = 24) were strongly associated with del(7q) and a higher 5-year survival rate compared with del(7q) without favorable cytogenetics (75% versus 46%, P = .03). Patients with -7 and inv(3),-5/del(5q), or + 21 had a 5-year survival rate of 5%. Stem cell transplantation analyzed as a time-dependent variable had no impact on overall survival. However, patients not achieving CR had a 31% survival rate after stem cell transplantation. Childhood AML with chromosome 7 aberrations represents a heterogeneous group of disorders with additional cytogenetic aberrations having a major prognostic impact which should be reflected in future risk-group stratification.
authors.affiliation: Department of Pediatrics, Aarhus University Hospital Skejby, Aarhus, Denmark. hasle@dadlnet.dk
authors.forename: Henrik
authors.initials: H
authors.lastname: Hasle
authors.forename: Todd A
authors.initials: TA
authors.lastname: Alonzo
authors.forename: Anne
authors.initials: A
authors.lastname: Auvrignon
authors.forename: Catherine
authors.initials: C
authors.lastname: Behar
authors.forename: Myron
authors.initials: M
authors.lastname: Chang
authors.forename: Ursula
authors.initials: U
authors.lastname: Creutzig
authors.forename: Alexandra
authors.initials: A
authors.lastname: Fischer
authors.forename: Erik
authors.initials: E
authors.lastname: Forestier
authors.forename: Alcira
authors.initials: A
authors.lastname: Fynn
authors.forename: Oskar A
authors.initials: OA
authors.lastname: Haas
authors.forename: Jochen
authors.initials: J
authors.lastname: Harbott
authors.forename: Christine J
authors.initials: CJ
authors.lastname: Harrison
authors.forename: Nyla A
authors.initials: NA
authors.lastname: Heerema
authors.forename: Marry M
authors.initials: MM
authors.lastname: van den Heuvel-Eibrink
authors.forename: Gertjan J L
authors.initials: GJ
authors.lastname: Kaspers
authors.forename: Franco
authors.initials: F
authors.lastname: Locatelli
authors.forename: Peter
authors.initials: P
authors.lastname: Noellke
authors.forename: Sophia
authors.initials: S
authors.lastname: Polychronopoulou
authors.forename: Yaddanapudi
authors.initials: Y
authors.lastname: Ravindranath
authors.forename: Bassem
authors.initials: B
authors.lastname: Razzouk
authors.forename: Dirk
authors.initials: D
authors.lastname: Reinhardt
authors.forename: Natalia N
authors.initials: NN
authors.lastname: Savva
authors.forename: Batia
authors.initials: B
authors.lastname: Stark
authors.forename: Stefan
authors.initials: S
authors.lastname: Suciu
authors.forename: Ichiro
authors.initials: I
authors.lastname: Tsukimoto
authors.forename: David K
authors.initials: DK
authors.lastname: Webb
authors.forename: Dorora
authors.initials: D
authors.lastname: Wojcik
authors.forename: William G
authors.initials: WG
authors.lastname: Woods
authors.forename: Martin
authors.initials: M
authors.lastname: Zimmermann
authors.forename: Charlotte M
authors.initials: CM
authors.lastname: Niemeyer
authors.forename: Susana C
authors.initials: SC
authors.lastname: Raimondi
mesh_terms: D000293:Adolescent; D002648:Child; D002869:Chromosome Aberrations; D002897:Chromosomes, Human, Pair 7; D005260:Female; D017353:Gene Deletion; D006801:Humans; D007391:International Cooperation; D015470:Leukemia, Myeloid, Acute / Q000235:genetics* / Q000401:mortality*; D008297:Male; D009006:Monosomy; D012074:Remission Induction; D012189:Retrospective Studies; D033581:Stem Cell Transplantation; D014178:Translocation, Genetic