Clinical and genetic features of pediatric acute lymphoblastic leukemia in Down syndrome in the Nordic countries.
Average rating
Cast your vote
You can rate an item by clicking the amount of stars they wish to award to this item.
When enough users have cast their vote on this item, the average rating will also be shown.
Star rating
Your vote was cast
Thank you for your feedback
Thank you for your feedback
Authors
Lundin, CatarinaForestier, 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
Issue Date
2014
Metadata
Show full item recordCitation
J Hematol Oncol. 2014, 7:32Abstract
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.
Description
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.Additional Links
http://dx.doi.org/ 10.1186/1756-8722-7-32http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4022076/
Rights
Archived with thanks to Journal of hematology & oncologyae974a485f413a2113503eed53cd6c53
10.1186/1756-8722-7-32
Scopus Count
Collections
Related articles
- Acute lymphoblastic leukemia and Down syndrome: presenting features and treatment outcome in the experience of the Italian Association of Pediatric Hematology and Oncology (AIEOP).
- Authors: Arico M, Ziino O, Valsecchi MG, Cazzaniga G, Baronci C, Messina C, Pession A, Santoro N, Basso G, Conter V, Italian Association of Pediatric Hematology and Oncology (AIEOP).
- Issue date: 2008 Aug 1
- Acute leukaemia in children with Down syndrome: a population-based Nordic study.
- Authors: Zeller B, Gustafsson G, Forestier E, Abrahamsson J, Clausen N, Heldrup J, Hovi L, Jonmundsson G, Lie SO, Glomstein A, Hasle H, Nordic Society of Paediatric Haematology and Oncology (NOPHO).
- Issue date: 2005 Mar
- Prognostic impact of karyotypic findings in childhood acute lymphoblastic leukaemia: a Nordic series comparing two treatment periods. For the Nordic Society of Paediatric Haematology and Oncology (NOPHO) Leukaemia Cytogenetic Study Group.
- Authors: Forestier E, Johansson B, Gustafsson G, Borgström G, Kerndrup G, Johannsson J, Heim S
- Issue date: 2000 Jul
- Acute lymphoblastic leukemia in children with Down syndrome: a retrospective analysis from the Ponte di Legno study group.
- Authors: Buitenkamp TD, Izraeli S, Zimmermann M, Forestier E, Heerema NA, van den Heuvel-Eibrink MM, Pieters R, Korbijn CM, Silverman LB, Schmiegelow K, Liang DC, Horibe K, Arico M, Biondi A, Basso G, Rabin KR, Schrappe M, Cario G, Mann G, Morak M, Panzer-Grümayer R, Mondelaers V, Lammens T, Cavé H, Stark B, Ganmore I, Moorman AV, Vora A, Hunger SP, Pui CH, Mullighan CG, Manabe A, Escherich G, Kowalczyk JR, Whitlock JA, Zwaan CM
- Issue date: 2014 Jan 2
- Clinical characteristics and outcome of children with Down syndrome and acute lymphoblastic leukemia: a Children's Cancer Group study.
- Authors: Whitlock JA, Sather HN, Gaynon P, Robison LL, Wells RJ, Trigg M, Heerema NA, Bhatia S
- Issue date: 2005 Dec 15