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Clinical and genetic features of pediatric acute lymphoblastic leukemia in Down syndrome in the Nordic countries.

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Authors
Lundin, Catarina
Forestier, Erik
Klarskov Andersen, Mette
Autio, Kirsi
Barbany, Gisela
Cavelier, Lucia
Golovleva, Irina
Heim, Sverre
Heinonen, Kristiina
Hovland, Randi
Johannsson, Johann H
Kjeldsen, Eigil
Nordgren, Ann
Palmqvist, Lars
Johansson, Bertil
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Issue Date
2014

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J Hematol Oncol. 2014, 7:32
Abstract
Children with Down syndrome (DS) have an increased risk for acute lymphoblastic leukemia (ALL). Although previous studies have shown that DS-ALL differs clinically and genetically from non-DS-ALL, much remains to be elucidated as regards genetic and prognostic factors in DS-ALL.
To address clinical and genetic differences between DS-ALL and non-DS-ALL and to identify prognostic factors in DS-ALL, we ascertained and reviewed all 128 pediatric DS-ALL diagnosed in the Nordic countries between 1981 and 2010. Their clinical and genetic features were compared with those of the 4,647 B-cell precursor (BCP) ALL cases diagnosed during the same time period.
All 128 DS-ALL were BCP ALL, comprising 2.7% of all such cases. The 5-year event-free survival (EFS) and overall survival (OS) were significantly (P = 0.026 and P = 0.003, respectively) worse for DS-ALL patients with white blood cell counts ≥50 × 109/l. The age distributions varied between the DS and non-DS cases, with age peaks at 2 and 3 years, respectively; none of the DS patients had infant ALL (P = 0.029). The platelet counts were lower in the DS-ALL group (P = 0.005). Abnormal karyotypes were more common in non-DS-ALL (P < 0.0001), and there was a significant difference in the modal number distribution, with only 2% high hyperdiploid DS-ALL cases (P < 0.0001). The 5-year EFS and 5-year OS were significantly worse for DS-ALL (0.574 and 0.691, respectively) compared with non-DS-ALL (0.783 and 0.894, respectively) in the NOPHO ALL-1992/2000 protocols (P < 0.001).
The present study adds further support for genetic and clinical differences between DS-ALL and non-DS-ALL.
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To access publisher's full text version of this article, please click on the hyperlink in Additional Links field or click on the hyperlink at the top of the page marked Files. This article is open access.
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http://dx.doi.org/ 10.1186/1756-8722-7-32
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4022076/
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Archived with thanks to Journal of hematology & oncology
ae974a485f413a2113503eed53cd6c53
10.1186/1756-8722-7-32
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