34 research outputs found
Genotype-phenotype correlation in cases of juvenile myelomonocytic leukemia with clonal RAS mutations
Aberrant DNA Methylation characterizes a subtype of juvenile myelomonocytic leukemia (JMML) with poor outcome
HLA-indentical umbilical cord blood transplantaton from a sibling donor in juvenile myelomonocytic leukemia
Genotype-phenotype correlation in cases of juvenile myelomonocytic leukemia with clonal RAS mutations.
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HLA-identical umbilical cord blood transplantation from a sibling donor in juvenile myelomonocytic leukemia
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