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

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 versus ALL need further evaluation and represent a challenge for the coming years.