Advanced

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

Lundin, Catarina LU ; Forestier, Erik; Andersen, Mette Klarskov; Autio, Kirsi; Barbany, Gisela; Cavelier, Lucia; Golovleva, Irina; Heim, Sverre; Heinonen, Kristiina and Hovland, Randi, et al. (2014) In Journal of Hematology & Oncology 7.
Abstract
Background: 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. Methods: 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. Results: All 128 DS-ALL were BCP ALL, comprising 2.7% of all such cases.... (More)
Background: 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. Methods: 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. Results: 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 x 10(9)/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). Conclusions: The present study adds further support for genetic and clinical differences between DS-ALL and non-DS-ALL. (Less)
Please use this url to cite or link to this publication:
author
, et al. (More)
(Less)
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Down syndrome, ALL, Children, Clinical features, Genetic features, Prognosis
in
Journal of Hematology & Oncology
volume
7
publisher
BioMed Central
external identifiers
  • wos:000336058600001
  • scopus:84899990860
ISSN
1756-8722
DOI
10.1186/1756-8722-7-32
language
English
LU publication?
yes
id
fa36fcd8-df19-418d-847c-d4d6964535b4 (old id 4470156)
date added to LUP
2014-07-01 07:40:11
date last changed
2017-07-30 04:05:47
@article{fa36fcd8-df19-418d-847c-d4d6964535b4,
  abstract     = {Background: 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. Methods: 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. Results: 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 &gt;= 50 x 10(9)/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 &lt; 0.0001), and there was a significant difference in the modal number distribution, with only 2% high hyperdiploid DS-ALL cases (P &lt; 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 &lt; 0.001). Conclusions: The present study adds further support for genetic and clinical differences between DS-ALL and non-DS-ALL.},
  articleno    = {32},
  author       = {Lundin, Catarina and Forestier, Erik and Andersen, Mette Klarskov and Autio, Kirsi and Barbany, Gisela and Cavelier, Lucia and Golovleva, Irina and Heim, Sverre and Heinonen, Kristiina and Hovland, Randi and Johannsson, Johann H. and Kjeldsen, Eigil and Nordgren, Ann and Palmqvist, Lars and Johansson, Bertil},
  issn         = {1756-8722},
  keyword      = {Down syndrome,ALL,Children,Clinical features,Genetic features,Prognosis},
  language     = {eng},
  publisher    = {BioMed Central},
  series       = {Journal of Hematology & Oncology},
  title        = {Clinical and genetic features of pediatric acute lymphoblastic leukemia in Down syndrome in the Nordic countries},
  url          = {http://dx.doi.org/10.1186/1756-8722-7-32},
  volume       = {7},
  year         = {2014},
}