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Clinical Trial
. 2007 Aug 15;110(4):1112-5.
doi: 10.1182/blood-2006-07-038299. Epub 2007 May 1.

Outcome of treatment in children with hypodiploid acute lymphoblastic leukemia

James B Nachman  1 , Nyla A Heerema, Harland Sather, Bruce Camitta, Erik Forestier, Christine J Harrison, Nicole Dastugue, Martin Schrappe, Ching-Hon Pui, Giuseppe Basso, Lewis B Silverman, Gritta E Janka-Schaub
Affiliations
Clinical Trial

Outcome of treatment in children with hypodiploid acute lymphoblastic leukemia

James B Nachman et al. Blood. .

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.

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Figures

Figure 1
Figure 1
Distribution of modal chromosome numbers in hypodiploid cases with fewer than 44 chromosomes. There are no patients with 30, 31, 32, or 42 chromosomes. For 2 patients, modal chromosome number could not be accurately determined.
Figure 2
Figure 2
EFS and OS for non-Ph+ hypodiploid patients.
Figure 3
Figure 3
Comparison of EFS for non-Ph+ hypodiploid patients with 44 chromosomes or fewer than 44 chromosomes.
Figure 4
Figure 4
Comparison of survival for non-Ph+ hypodiploid patients with 44 chromosomes or fewer than 44 chromosomes.
Figure 5
Figure 5
EFS for 130 evaluable, non-Ph+ patients by modal chromosome number: 44 chromosomes, 40 to 43 chromosomes, 30 to 39 chromosomes, and 24 to 29 chromosomes.
Figure 6
Figure 6
OS for 130 evaluable, non-Ph+ patients by modal chromosome number: 44 chromosomes, 40 to 43 chromosomes, 30 to 39 chromosomes, and 24 to 29 chromosomes.
See this image and copyright information in PMC

References

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