Down's syndrome and acute lymphoblastic leukaemia: clinical features and response to treatment
- aMolecular Haematology Unit, Camelia Botnar Laboratories, Institute of Child Health, 30 Guilford Street, London WCIN 1EH, UK, bClinical Trial Service Unit, Harkness Building, Radcliffe Infirmary, Oxford OX2 6HE, UK, cNorthern and Yorkshire Region, Research School of Medicine, 24 Hyde Terrace, Leeds LS2 9LN, UK, dBirmingham Children's Hospital, Steelhouse Lane, Birmingham B4 6NH, UK, eRoyal Hospital for Sick Children, Glasgow G3 8SJ, UK, fHospital for Sick Children, Great Ormond Street, London WC1N 3JH, UK
- Dr Chessellsj.chessells{at}ich.ucl.ac.uk
- Accepted 6 July 2001
Abstract
AIMS To examine the clinical and biological features of acute lymphoblastic leukaemia in children with Down's syndrome (DS), to compare their survival with other children, and to determine if entry to trials and survival has improved.
METHODS Examination of presenting features and response to treatment in patients treated in two consecutive national trials, MRC UKALL X and XI.
RESULTS The proportion of children with DS was significantly higher in UKALL XI (1.9%) than UKALL X (0.9%). Children with DS tended to be under 10 years and to have the common ALL subtype. Cytogenetic analysis showed that favourable features, such as high hyperdiploidy and t(12;21) were less frequent but also that there was a lack of translocations associated with a poor prognosis. Children with DS showed no increase in risk of relapse at any site but their survival and event free survival were inferior to other children. These results were caused by an increased number of infective deaths during remission (11% compared to 2%). At five years overall survival was 73% in DS children compared with 82% in other children; event free survival was 53% compared to 63% in non-DS children.
CONCLUSIONS Entry of children with DS to national trials has increased and survival has improved. However they remain at risk of relapse and also of treatment related mortality. These findings emphasise the need for both intensive chemotherapy and optimal supportive care.
Footnotes
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A report from the MRC childhood leukaemia working party
