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Outcome of treatment in children with hypodiploid acute lymphoblastic leukemia. Blood 2007 Aug 15;110(4):1112-5

Date

05/03/2007

Pubmed ID

17473063

Pubmed Central ID

PMC1939895

DOI

10.1182/blood-2006-07-038299

Abstract

One-hundred thirty-nine patients with acute lymphoblastic leukemia (ALL) and hypodiploidy (fewer than 45 chromosomes) were collected from 10 different national ALL study groups and single institutions. Patients were stratified by modal chromosome number into 4 groups: 24 to 29 (N = 46); 33 to 39 (N = 26); 40 to 43 (N = 13); and 44 (N = 54) chromosomes. Nine patients were Philadelphia chromosome (Ph) positive (4 cases: 44 chromosomes; 5 cases: 40-43 chromosomes) and were not considered further. Event-free survival (EFS) and overall survival (OS) of the remaining 130 patients were 38.5% +/- 4.4% and 49.8% +/- 4.2% at 8 years, respectively. There were no significant differences in outcome between patients with 24 to 29, 33 to 39, or 40 to 43 chromosomes. Compared with patients with fewer than 44 chromosomes, patients with 44 chromosomes had a significantly better EFS (P = .01; 8-year estimate, 52.2% vs 30.1%) and OS (P = .017; 69% vs 37.5%). For patients with 44 chromosomes, monosomy 7, the presence of a dicentric chromosome, or both predicted a worse EFS but similar OS. Doubling of the hypodiploid clone occurred in 32 patients (24-29 chromosomes [n = 25] and 33-39 chromosomes [n = 7]) and had no prognostic implication. Children and adolescents with ALL and hypodiploidy with fewer than 44 chromosomes have a poor outcome despite contemporary therapy.

Author List

Nachman JB, Heerema NA, Sather H, Camitta B, Forestier E, Harrison CJ, Dastugue N, Schrappe M, Pui CH, Basso G, Silverman LB, Janka-Schaub GE

Author

Bruce M. Camitta MD Adjunct Professor in the Medicine department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Bone Marrow Transplantation
Child
Child, Preschool
Female
Humans
Infant
Karyotyping
Male
Medical Records
Philadelphia Chromosome
Ploidies
Precursor Cell Lymphoblastic Leukemia-Lymphoma
Prognosis
Retrospective Studies
Survival Rate