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Acute leukaemia in children with Down syndrome: a population-based Nordic study

Zeller, B; Gustafsson, G; Forestier, E; Abrahamsson, J; Clausen, N; Heldrup, Jesper LU ; Hovi, L; Jonmundsson, G; Lie, S O and Glomstein, A, et al. (2005) In British Journal of Haematology 128(6). p.797-804
Abstract
To determine the epidemiology and outcome of children with Down syndrome (DS) diagnosed with acute leukaemia in the Nordic countries, data registered in the Nordic Society of Paediatric Haematology and Oncology (NOPHO) population-based leukaemia registry were analysed. Of 3494 children with acute leukaemia diagnosed between July 1984 and December 2001, 136 patients (3.9%) with DS were identified. 2.1% of the children with acute lymphoid leukaemia (ALL) and 14.0% of the children with acute myeloid leukaemia (AML) had DS. In ALL, DS patients had similar age and sex distribution and no major differences in blood counts compared with non-DS children. None of the DS patients had T cell leukaemia. Outcome was inferior to that of non-DS children... (More)
To determine the epidemiology and outcome of children with Down syndrome (DS) diagnosed with acute leukaemia in the Nordic countries, data registered in the Nordic Society of Paediatric Haematology and Oncology (NOPHO) population-based leukaemia registry were analysed. Of 3494 children with acute leukaemia diagnosed between July 1984 and December 2001, 136 patients (3.9%) with DS were identified. 2.1% of the children with acute lymphoid leukaemia (ALL) and 14.0% of the children with acute myeloid leukaemia (AML) had DS. In ALL, DS patients had similar age and sex distribution and no major differences in blood counts compared with non-DS children. None of the DS patients had T cell leukaemia. Outcome was inferior to that of non-DS children and treatment results did not improve over time. In AML, DS patients showed a significant female predominance and all but one were <5 years old. DS patients with AML had significantly lower platelet and white blood cell counts and two-thirds were type M7 as according to the French-American-British classification. None of the patients <5 years of age had typical AML cytogenetic aberrations. Outcome was far better in the DS group. DS patients treated for AML after 1992 had an excellent outcome (probability of event-free survival, 83 +/- 6%). The high proportion of female DS patients with AML is unexplained. The differing treatment results in AML versus ALL need further evaluation and represent a challenge for the coming years. (Less)
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publication status
published
subject
keywords
children, epidemiology, Down syndrome, acute lymphoid leukaemia, acute myeloid leukaemia
in
British Journal of Haematology
volume
128
issue
6
pages
797 - 804
publisher
Federation of European Neuroscience Societies and Blackwell Publishing Ltd
external identifiers
  • pmid:15755283
  • wos:000227480300007
  • scopus:20144388603
ISSN
0007-1048
DOI
10.1111/j.1365-2141.2005.05398.x
language
English
LU publication?
yes
id
0c8fa629-e217-4fdd-b2db-ad244b027380 (old id 250037)
date added to LUP
2007-08-06 13:10:01
date last changed
2017-07-30 03:45:37
@article{0c8fa629-e217-4fdd-b2db-ad244b027380,
  abstract     = {To determine the epidemiology and outcome of children with Down syndrome (DS) diagnosed with acute leukaemia in the Nordic countries, data registered in the Nordic Society of Paediatric Haematology and Oncology (NOPHO) population-based leukaemia registry were analysed. Of 3494 children with acute leukaemia diagnosed between July 1984 and December 2001, 136 patients (3.9%) with DS were identified. 2.1% of the children with acute lymphoid leukaemia (ALL) and 14.0% of the children with acute myeloid leukaemia (AML) had DS. In ALL, DS patients had similar age and sex distribution and no major differences in blood counts compared with non-DS children. None of the DS patients had T cell leukaemia. Outcome was inferior to that of non-DS children and treatment results did not improve over time. In AML, DS patients showed a significant female predominance and all but one were &lt;5 years old. DS patients with AML had significantly lower platelet and white blood cell counts and two-thirds were type M7 as according to the French-American-British classification. None of the patients &lt;5 years of age had typical AML cytogenetic aberrations. Outcome was far better in the DS group. DS patients treated for AML after 1992 had an excellent outcome (probability of event-free survival, 83 +/- 6%). The high proportion of female DS patients with AML is unexplained. The differing treatment results in AML versus ALL need further evaluation and represent a challenge for the coming years.},
  author       = {Zeller, B and Gustafsson, G and Forestier, E and Abrahamsson, J and Clausen, N and Heldrup, Jesper and Hovi, L and Jonmundsson, G and Lie, S O and Glomstein, A and Hasle, H},
  issn         = {0007-1048},
  keyword      = {children,epidemiology,Down syndrome,acute lymphoid leukaemia,acute myeloid leukaemia},
  language     = {eng},
  number       = {6},
  pages        = {797--804},
  publisher    = {Federation of European Neuroscience Societies and Blackwell Publishing Ltd},
  series       = {British Journal of Haematology},
  title        = {Acute leukaemia in children with Down syndrome: a population-based Nordic study},
  url          = {http://dx.doi.org/10.1111/j.1365-2141.2005.05398.x},
  volume       = {128},
  year         = {2005},
}