Complex karyotype newly defined: the strongest prognostic factor in advanced childhood myelodysplastic syndrome.
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.
Göhring
Gudrun
G
Institute of Cell and Molecular Pathology, Hannover Medical School, Hannover, Germany.
Michalova
Kyra
K
Beverloo
H Berna
HB
Betts
David
D
Harbott
Jochen
J
Haas
Oskar A
OA
Kerndrup
Gitte
G
Sainati
Laura
L
Bergstraesser
Eva
E
Hasle
Henrik
H
Stary
Jan
J
Trebo
Monika
M
van den Heuvel-Eibrink
Marry M
MM
Zecca
Marco
M
van Wering
Elisabeth R
ER
Fischer
Alexandra
A
Noellke
Peter
P
Strahm
Brigitte
B
Locatelli
Franco
F
Niemeyer
Charlotte M
CM
Schlegelberger
Brigitte
B
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