TY - JOUR
T1 - Complex karyotype newly defined
T2 - The strongest prognostic factor in advanced childhood myelodysplastic syndrome
AU - Göhring, Gudrun
AU - Michalova, Kyra
AU - Beverloo, H. Berna
AU - Betts, David
AU - Harbott, Jochen
AU - Haas, Oskar A.
AU - Kerndrup, Gitte
AU - Sainati, Laura
AU - Bergstraesser, Eva
AU - Hasle, Henrik
AU - Starý, Jan
AU - Trebo, Monika
AU - Van Den Heuvel-Eibrink, Marry M.
AU - Zecca, Marco
AU - Van Wering, Elisabeth R.
AU - Fischer, Alexandra
AU - Noellke, Peter
AU - Strahm, Brigitte
AU - Locatelli, Franco
AU - Niemeyer, Charlotte M.
AU - Schlegelberger, Brigitte
PY - 2010/11/11
Y1 - 2010/11/11
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=78149432321&partnerID=8YFLogxK
U2 - 10.1182/blood-2010-04-280313
DO - 10.1182/blood-2010-04-280313
M3 - Article
C2 - 20802024
AN - SCOPUS:78149432321
SN - 0006-4971
VL - 116
SP - 3766
EP - 3769
JO - Blood
JF - Blood
IS - 19
ER -