Acute leukaemia in children with Down syndrome: a population-based Nordic study
(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)
Please use this url to cite or link to this publication:
https://lup.lub.lu.se/record/250037
- author
- organization
- publishing date
- 2005
- type
- Contribution to journal
- 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
- Wiley-Blackwell
- 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
- 2016-04-01 12:13:27
- date last changed
- 2022-08-13 20:44:17
@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 <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.}}, 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}}, keywords = {{children; epidemiology; Down syndrome; acute lymphoid leukaemia; acute myeloid leukaemia}}, language = {{eng}}, number = {{6}}, pages = {{797--804}}, publisher = {{Wiley-Blackwell}}, 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}}, doi = {{10.1111/j.1365-2141.2005.05398.x}}, volume = {{128}}, year = {{2005}}, }