title: High modal number and triple trisomies are highly correlated favorable factors in childhood B-cell precursor high hyperdiploid acute lymphoblastic leukemia treated according to the NOPHO ALL 1992/2000 protocols.
abstract: Between 1992 and 2008, 713 high hyperdiploid acute lymphoblastic leukemias in children aged 1-15 years were diagnosed and treated according to the Nordic Society for Pediatric Hematology and Oncology acute lymphoblastic leukemia 1992/2000 protocols. Twenty (2.8%) harbored t(1;19), t(9;22), der(11q23), or t(12;21). The median age of patients with "classic" high hyperdiploidy was lower than that of patients with translocation-positive high hyperdiploidy (P<0.001). Cases with triple trisomies (+4, +10, +17), comprising 50%, had higher modal numbers than the triple trisomy-negative cases (P<0.0001). The probabilities of event-free survival and overall survival were lower for those with white blood cell counts ≥ 50 × 10(9)/L (P=0.017/P=0.009), ≥ 5% bone marrow blasts at day 29 (P=0.001/0.002), and for high-risk patients (P<0.001/P=0.003), whereas event-free, but not overall, survival, was higher for cases with gains of chromosomes 4 (P<0.0001), 6 (P<0.003), 17 (P=0.010), 18 (P=0.049), and 22 (P=0.040), triple trisomies (P=0.002), and modal numbers >53/55 (P=0.020/0.024). In multivariate analyses, modal number and triple trisomies were significantly associated with superior event-free survival in separate analyses with age and white blood cell counts. When including both modal numbers and triple trisomies, only low white blood cell counts were significantly associated with superior event-free survival (P=0.009). We conclude that high modal chromosome numbers and triple trisomies are highly correlated prognostic factors and that these two parameters identify the same subgroup of patients characterized by a particularly favorable outcome.
authors: Division of Clinical Genetics, Department of Laboratory Medicine, Lund University, Lund, Sweden. kajsa.paulsson@med.lu.se
authors: Kajsa
authors: K
authors: Paulsson
authors: Erik
authors: E
authors: Forestier
authors: Mette K
authors: MK
authors: Andersen
authors: Kirsi
authors: K
authors: Autio
authors: Gisela
authors: G
authors: Barbany
authors: Georg
authors: G
authors: Borgström
authors: Lucia
authors: L
authors: Cavelier
authors: Irina
authors: I
authors: Golovleva
authors: Sverre
authors: S
authors: Heim
authors: Kristiina
authors: K
authors: Heinonen
authors: Randi
authors: R
authors: Hovland
authors: Johann H
authors: JH
authors: Johannsson
authors: Eigil
authors: E
authors: Kjeldsen
authors: Ann
authors: A
authors: Nordgren
authors: Lars
authors: L
authors: Palmqvist
authors: Bertil
authors: B
authors: Johansson
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D011159:Population Surveillance / Q000379:methods; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000235:genetics* / Q000401:mortality / Q000628:therapy*; D054448:Precursor Cells, B-Lymphoid / Q000502:physiology*; D011446:Prospective Studies; D015996:Survival Rate / Q000639:trends; D016896:Treatment Outcome; D014314:Trisomy / Q000235:genetics*
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