title: Trisomy 8 in pediatric acute myeloid leukemia: A NOPHO-AML study.
abstract: Trisomy 8 (+8) is a common cytogenetic aberration in acute myeloid leukemia (AML); however, the impact of +8 in pediatric AML is largely unknown. We retrospectively investigated 609 patients from the NOPHO-AML database to determine the clinical and cytogenetic characteristics of +8 in pediatric AML and to investigate its prognostic impact. Complete cytogenetic data were available in 596 patients (98%) aged 0-18 years, diagnosed from 1993 to 2012, and treated according to the NOPHO-AML 1993 and 2004 protocols in the Nordic countries and Hong Kong. We identified 86 patients (14%) with +8. Trisomy 8 was combined with other cytogenetic aberrations in 68 patients (11%) (+8 other) and in 18 patients (3%), it was the sole abnormality (+8 alone). Trisomy 8 was associated with FAB M5 (36%) but otherwise clinically comparable with non-trisomy 8 patients. Trisomy 8 was favorable in patients of young age and with t(9;11). Trisomy 8 alone was associated with older age (median age 10.1 years), FAB M2 (33%), and FLT3-ITD mutations (58%). The 5-year event-free survival for patients with +8 alone was 50% and 5-year overall survival was 75%. In conclusion, +8 is one of the most common cytogenetic aberrations in pediatric AML. Trisomy 8 positive AML is a heterogeneous group and the majority of cases have additional cytogenetic aberrations. Patients with +8 alone differed from patients with +8 other and were associated with older age, FAB M2, and FLT3-ITD aberrations. There were no differences in survival despite the more frequent occurrence of FLT3-ITD in +8 alone. © 2016 Wiley Periodicals, Inc.
authors.affiliation: Department of Pediatrics, Aarhus University Hospital, Aarhus, Denmark.
authors.forename: Anne Cathrine Lund
authors.identifier: 0000-0001-5298-0803
authors.initials: AC
authors.lastname: Laursen
authors.affiliation: Department of Pediatrics, Aarhus University Hospital, Aarhus, Denmark.
authors.forename: Julie Damgaard
authors.initials: JD
authors.lastname: Sandahl
authors.affiliation: Cancer Cytogenetics Laboratory, Department of Hematology, Aarhus University Hospital, Aarhus, Denmark.
authors.forename: Eigil
authors.initials: E
authors.lastname: Kjeldsen
authors.affiliation: Institution for Clinical Sciences, Department of Pediatrics, Queen Silvia Children's Hospital, Gothenburg, Sweden.
authors.forename: Jonas
authors.initials: J
authors.lastname: Abrahamsson
authors.affiliation: Department of Pediatrics, Aarhus University Hospital, Aarhus, Denmark.
authors.forename: Peter
authors.initials: P
authors.lastname: Asdahl
authors.affiliation: Department of Pediatrics, Queen Mary Hospital and Hong Kong Pediatric Hematology & Oncology Study Group (HKPHOSG), Hong Kong, China.
authors.forename: Shau-Yin
authors.initials: SY
authors.lastname: Ha
authors.affiliation: Department of Pediatrics, University Hospital, Lund, Sweden.
authors.forename: Jesper
authors.initials: J
authors.lastname: Heldrup
authors.affiliation: Children's Hospital, Helsinki University Central Hospital, University of Helsinki, Helsinki, Finland.
authors.forename: Kirsi
authors.initials: K
authors.lastname: Jahnukainen
authors.affiliation: Department of Pediatrics, Landspitalinn, Reykjavik, Iceland.
authors.forename: Ólafur G
authors.initials: ÓG
authors.lastname: Jónsson
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark.
authors.forename: Birgitte
authors.initials: B
authors.lastname: Lausen
authors.affiliation: Department of Woman's and Children's Health, Uppsala University, Uppsala, Sweden.
authors.forename: Josefine
authors.initials: J
authors.lastname: Palle
authors.affiliation: Department of Pediatric Medicine, Oslo University Hospital, Oslo, Norway.
authors.forename: Bernward
authors.initials: B
authors.lastname: Zeller
authors.affiliation: Department of Medical Biosciences, Clinical Genetics, Umeå University Hospital, Umeå, Sweden.
authors.forename: Erik
authors.initials: E
authors.lastname: Forestier
authors.affiliation: Department of Pediatrics, Aarhus University Hospital, Aarhus, Denmark.
authors.forename: Henrik
authors.initials: H
authors.lastname: Hasle
mesh_terms: D000293:Adolescent; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D002869:Chromosome Aberrations; D002898:Chromosomes, Human, Pair 8 / Q000235:genetics; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D015470:Leukemia, Myeloid, Acute / Q000235:genetics*; D008297:Male; D009367:Neoplasm Staging; D011379:Prognosis; D015996:Survival Rate; D020080:Tandem Repeat Sequences / Q000235:genetics*; D014314:Trisomy / Q000235:genetics*; D051941:fms-Like Tyrosine Kinase 3 / Q000235:genetics*
chemical_list: D051941:fms-Like Tyrosine Kinase 3