title: Hyperdiploidy with 58-66 chromosomes in childhood B-acute lymphoblastic leukemia is highly curable: 58951 CLG-EORTC results.
abstract: The aim of our study was to analyze the factors contributing to heterogeneity of prognosis in patients with hyperdiploidy>50 chromosomes (HD>50), a group of B-cell precursor acute lymphoblastic leukemia with favorable outcome. The 541 HD>50 patients registered prospectively in the 58951 European Organisation for Research and Treatment of Cancer (EORTC) Children's Leukemia Group (CLG) trial, identified by karyotype (446 patients) and by DNA index (DI) (490 patients), had a 6-year event-free survival (EFS) of 89.0% (standard error [SE] = 1.5%) and a 6-year overall survival (OS) of 95.9% (SE = 0.9%). The strongest prognostic factor was the modal number of chromosomes (MNC): the 6-year EFS of 51-53, 54-57, and 58-66 MNC groups were 80%, 89%, and 99%, respectively (P < .0001). Ploidy assessed by DI was also a favorable factor: the higher the DI, the better the outcome. The 6-year EFS of the 3 subgroups of DI < 1.16/≥1.16-<1.24/≥1.24 were 83%, 90%, and 95%, respectively (P = .009). All usual combinations of trisomies (chromosomes 4, 10, 17, 18) were significant favorable factors but had lower EFS when MNC was lower than 58. In multivariate analysis, MNC remained the strongest factor. Consequently, the best indicator for excellent outcome was ploidy assessed by karyotype because patients with 58-66 chromosomes stood every chance of being cured (OS of 100% at 6-year follow-up) with less-intensive therapy. This trial was registered at www.clinicaltrials.gov as #NCT00003728. Registered: http://www.eortc.org/, http://clinicaltrials.gov/show/NCT00003728.
authors: Hematology Laboratory, Purpan Hospital, Toulouse, France. dastugue.n@chu-toulouse.fr
authors: Nicole
authors: N
authors: Dastugue
authors: Stefan
authors: S
authors: Suciu
authors: Geneviève
authors: G
authors: Plat
authors: Frank
authors: F
authors: Speleman
authors: Hélène
authors: H
authors: Cavé
authors: Sandrine
authors: S
authors: Girard
authors: Marleen
authors: M
authors: Bakkus
authors: Marie Pierre
authors: MP
authors: Pagès
authors: Karima
authors: K
authors: Yakouben
authors: Brigitte
authors: B
authors: Nelken
authors: Anne
authors: A
authors: Uyttebroeck
authors: Carine
authors: C
authors: Gervais
authors: Patrick
authors: P
authors: Lutz
authors: Manuel R
authors: MR
authors: Teixeira
authors: Pierre
authors: P
authors: Heimann
authors: Alice
authors: A
authors: Ferster
authors: Pierre
authors: P
authors: Rohrlich
authors: Marie Agnès
authors: MA
authors: Collonge
authors: Martine
authors: M
authors: Munzer
authors: Isabelle
authors: I
authors: Luquet
authors: Patrick
authors: P
authors: Boutard
authors: Nicolas
authors: N
authors: Sirvent
authors: Matthias
authors: M
authors: Karrasch
authors: Yves
authors: Y
authors: Bertrand
authors: Yves
authors: Y
authors: Benoit
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D002869:Chromosome Aberrations / Q000706:statistics & numerical data; D002875:Chromosomes / Q000235:genetics; D002986:Clinical Trials as Topic / Q000379:methods; D004171:Diploidy; D005260:Female; D005500:Follow-Up Studies; D018740:Genetic Heterogeneity; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007621:Karyotyping; D008297:Male; D011123:Polyploidy; D015452:Precursor B-Cell Lymphoblastic Leukemia-Lymphoma / Q000453:epidemiology / Q000235:genetics* / Q000473:pathology / Q000628:therapy*; D012074:Remission Induction