title: Complex karyotype newly defined: the strongest prognostic factor in advanced childhood myelodysplastic syndrome.
abstract: To identify cytogenetic risk factors predicting outcome in children with advanced myelodysplastic syndrome, overall survival of 192 children prospectively enrolled in European Working Group of Myelodysplastic Syndrome in Childhood studies was evaluated with regard to karyotypic complexity. Structurally complex constitutes a new definition of complex karyotype characterized by more than or equal to 3 chromosomal aberrations, including at least one structural aberration. Five-year overall survival in patients with more than or equal to 3 clonal aberrations, which were not structurally complex, did not differ from that observed in patients with normal karyotype. Cox regression analysis revealed the presence of a monosomal and structurally complex karyotype to be strongly associated with poor prognosis (hazard ratio = 4.6, P < .01). Notably, a structurally complex karyotype without a monosomy was associated with a very short 2-year overall survival probability of only 14% (hazard ratio = 14.5; P < .01). The presence of a structurally complex karyotype was the strongest independent prognostic marker predicting poor outcome in children with advanced myelodysplastic syndrome.
authors.affiliation: Institute of Cell and Molecular Pathology, Hannover Medical School, Hannover, Germany.
authors.forename: Gudrun
authors.initials: G
authors.lastname: Göhring
authors.forename: Kyra
authors.initials: K
authors.lastname: Michalova
authors.forename: H Berna
authors.initials: HB
authors.lastname: Beverloo
authors.forename: David
authors.initials: D
authors.lastname: Betts
authors.forename: Jochen
authors.initials: J
authors.lastname: Harbott
authors.forename: Oskar A
authors.initials: OA
authors.lastname: Haas
authors.forename: Gitte
authors.initials: G
authors.lastname: Kerndrup
authors.forename: Laura
authors.initials: L
authors.lastname: Sainati
authors.forename: Eva
authors.initials: E
authors.lastname: Bergstraesser
authors.forename: Henrik
authors.initials: H
authors.lastname: Hasle
authors.forename: Jan
authors.initials: J
authors.lastname: Stary
authors.forename: Monika
authors.initials: M
authors.lastname: Trebo
authors.forename: Marry M
authors.initials: MM
authors.lastname: van den Heuvel-Eibrink
authors.forename: Marco
authors.initials: M
authors.lastname: Zecca
authors.forename: Elisabeth R
authors.initials: ER
authors.lastname: van Wering
authors.forename: Alexandra
authors.initials: A
authors.lastname: Fischer
authors.forename: Peter
authors.initials: P
authors.lastname: Noellke
authors.forename: Brigitte
authors.initials: B
authors.lastname: Strahm
authors.forename: Franco
authors.initials: F
authors.lastname: Locatelli
authors.forename: Charlotte M
authors.initials: CM
authors.lastname: Niemeyer
authors.forename: Brigitte
authors.initials: B
authors.lastname: Schlegelberger
mesh_terms: D000293:Adolescent; D000754:Anemia, Refractory, with Excess of Blasts / Q000235:genetics / Q000401:mortality / Q000628:therapy; D002648:Child; D002869:Chromosome Aberrations; D020732:Cytogenetic Analysis; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D053208:Kaplan-Meier Estimate; D007621:Karyotyping; D008297:Male; D009006:Monosomy; D009190:Myelodysplastic Syndromes / Q000235:genetics* / Q000401:mortality / Q000628:therapy; D011379:Prognosis; D011446:Prospective Studies; D012307:Risk Factors