Outcome heterogeneity in childhood high-hyperdiploid acute lymphoblastic leukemia.

Journal article

High hyperdiploidy (HeH) (51 to 65 chromosomes) is found in one third of children with acute lymphoblastic leukemia and is associated with a good prognosis. Cytogenetic features may further refine this prognosis and identify patients with a poor outcome. We examined the effect of sex, age, individual trisomies, modal number, and structural abnormalities on survival among 700 children with HeH. Univariate analysis showed that age. sex, +4, +10, +18, and a high modal number were associated with survival. Multivariate analysis however, revealed that only age, sex, +4, and +18 were independent indicators. Hazard scores for predicting relapse and mortality were constructed. Three risk groups with 5-year event-free survival (EFS) rates of 86%, 75%, and 50% (P <.0001) were identified. The high-risk group comprised boys older than 9 years, boys aged 1 through 9 years without +18, and girls older than 9 years without +18, while girls aged 1 through 9 years with +18 had the best EFS. In terms of mortality, those younger than age 10 years with both +4 and +18 had an improved survival (96% vs 84% at 5 years, P <.0001). These findings confirm that the outcome of children with HeH is heterogeneous and that specific trisomies can identify patients with the greatest and least risk of treatment failure.

Authors: Moorman, A; Richards, S; Martineau, M; Cheung, K; Robinson, H

• Publication status: Published
• Journal: Blood
• Volume: 102
• Issue: 8
• Pages: 2756-2762
• Publication date: 2003-10-01
• DOI: 10.1182/blood-2003-04-1128
• EISSN: 1528-0020
• ISSN: 0006-4971
• Language: English
• Keywords: Proportional Hazards Models; United Kingdom Medical Research Council's Childhood Leukemia Working Party; Infant; Diploidy; Disease-Free Survival; Precursor Cell Lymphoblastic Leukemia-Lymphoma; Time Factors; Adolescent; Female; Humans; Karyotyping; In Situ Hybridization, Fluorescence; Sex Factors; Prognosis; Multivariate Analysis; Male; Child, Preschool; Treatment Outcome; Child; Age Factors; Trisomy