title: Prognostic impact of specific chromosomal aberrations in a large group of pediatric patients with acute myeloid leukemia treated uniformly according to trial AML-BFM 98.
abstract: OBJECTIVE
  Because cytogenetic data are essential for risk stratification of childhood acute myeloid leukemia (AML), the impact of chromosomal aberrations is crucial.
  
  
  METHODS
  Data of a large group of patients younger than 18 years treated according to study AML-Berlin-Frankfurt-Münster (BFM) 98 (n = 454), including their cytogenetics, were analyzed.
  
  
  RESULTS
  The favorable outcome in the subgroups of patients with t(8;21), inv(16), and t(15;17), with an overall survival of 91% (SE, 4%), 92% (SE, 6%), and 87% (SE, 5%), respectively, was confirmed. Within this group, the 5-year probability of event-free survival (pEFS) of all 17 children with t(8;21) and additional aberrations apart from del(9q) or -X/-Y was 100%. As expected, the cytogenetic finding of a complex karyotype (n = 35; pEFS, 33%; SE, 8%) or a monosomy 7 (n = 12; pEFS, 17%; SE, 11%) was associated with a poor outcome. Compared with remaining patients with cytogenetic data (pEFS, 48%; SE, 2%), prognosis in patients with an MLL rearrangement (n = 91) was inferior (pEFS, 34%; SE, 5%; P = .0005). Particularly, children with t(9;11) and additional aberrations (n = 13; pEFS, 31%; SE, 14%) and MLL rearrangements other than t(9;11) and t(11;19) (n = 41; pEFS, 24%; SE, 7%) had an unfavorable outcome. Nine patients with aberrations in 12p showed an adverse prognosis (pEFS, 11%; SE, 10%). The outcome of patients with aberrations of chromosome 5 (n = 13) was better than expected (pEFS, 50%; SE, 13%).
  
  
  CONCLUSIONS
  Because the prognostic value of rare recurrent chromosomal aberrations still has to be elucidated, these data will contribute to future risk stratification for the treatment of pediatric AML.
authors.affiliation: Pediatric Hematology and Oncology, Medical School Hannover, Carl-Neuberg-Strasse 1, Hannover, Germany. neuhoff.christine.von@mh-hannover.de
authors.forename: Christine
authors.initials: C
authors.lastname: von Neuhoff
authors.forename: Dirk
authors.initials: D
authors.lastname: Reinhardt
authors.forename: Annette
authors.initials: A
authors.lastname: Sander
authors.forename: Martin
authors.initials: M
authors.lastname: Zimmermann
authors.forename: Jutta
authors.initials: J
authors.lastname: Bradtke
authors.forename: David R
authors.initials: DR
authors.lastname: Betts
authors.forename: Zuzana
authors.initials: Z
authors.lastname: Zemanova
authors.forename: Jan
authors.initials: J
authors.lastname: Stary
authors.forename: Jean-Pierre
authors.initials: JP
authors.lastname: Bourquin
authors.forename: Oskar A
authors.initials: OA
authors.lastname: Haas
authors.forename: Michael N
authors.initials: MN
authors.lastname: Dworzak
authors.forename: Ursula
authors.initials: U
authors.lastname: Creutzig
mesh_terms: D000293:Adolescent; D000367:Age Factors; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage*; D002648:Child; D002675:Child, Preschool; D002869:Chromosome Aberrations; D002891:Chromosomes, Human, Pair 21; D002898:Chromosomes, Human, Pair 8; D003131:Combined Modality Therapy; D016001:Confidence Intervals; D003561:Cytarabine / Q000008:administration & dosage; D020732:Cytogenetic Analysis; D018572:Disease-Free Survival; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015255:Idarubicin / Q000008:administration & dosage; D017404:In Situ Hybridization, Fluorescence; D015470:Leukemia, Myeloid, Acute / Q000175:diagnosis / Q000235:genetics* / Q000401:mortality / Q000628:therapy*; D008297:Male; D011336:Probability; D016016:Proportional Hazards Models; D018714:Radiotherapy, Adjuvant; D020133:Reverse Transcriptase Polymerase Chain Reaction; D018570:Risk Assessment; D012720:Severity of Illness Index; D018709:Statistics, Nonparametric; D033581:Stem Cell Transplantation; D016019:Survival Analysis; D014184:Transplantation, Homologous; D016896:Treatment Outcome
chemical_list: D003561:Cytarabine; D005047:Etoposide; D015255:Idarubicin