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A population-based study of childhood myelodysplastic syndrome in British Columbia, Canada
H Hasle1, L D Wadsworth, B G Massing
1Department of Paediatrics, Aarhus University Hospital Skejby, Aarhus, Denmark. hasle@post2.tele.dk
Insights
Childhood myelodysplastic syndrome (MDS) incidence is underestimated by cancer registries. This study found a higher incidence than previously reported, highlighting data inaccuracies in childhood leukemia research.
Area of Science:
- Pediatric Hematology Oncology
- Epidemiology
- Cancer Registries
Background:
- Childhood myelodysplastic syndrome (MDS) is considered rare, with conflicting incidence data from previous studies.
- Accurate epidemiological data is crucial for understanding childhood MDS and improving patient outcomes.
Purpose of the Study:
- To determine the population-based incidence of childhood MDS in British Columbia (BC) from 1982-1996.
- To evaluate the accuracy of cancer registry and treatment trial data for childhood MDS.
- To explore trends and associated conditions in childhood MDS.
Main Methods:
- Population-based study of children aged 0-14 years in BC.
- Data collection and analysis of diagnosed MDS cases between 1982 and 1996.
- Comparison of study findings with existing cancer registry and treatment trial data.
Main Results:
- An annual incidence of 3.2 per million children was observed for MDS, representing 6% of childhood leukemias.
- Cancer registry data significantly underestimated MDS incidence, with only one-third of cases correctly registered.
- Associated abnormalities, particularly Down syndrome, were present in 48% of MDS cases.
Conclusions:
- Childhood MDS incidence may be higher than previously estimated.
- Cancer registries and treatment-based studies provide inaccurate and underestimated data on childhood MDS.
- Further research is needed to improve data collection and ascertainment for childhood MDS.
Abstract:
Myelodysplastic syndrome (MDS) is considered to be very rare in children. However, the only two published population-based studies reported widely divergent incidence figures. To further explore the epidemiology of childhood MDS and to evaluate the accuracy of cancer registry and treatment trial data, we conducted a population-based study of children aged 0-14 years in British Columbia (BC), Canada, between 1982 and 1996. MDS was diagnosed in 31 cases corresponding to an annual incidence of 3.2 per million children or 6% of all leukaemias, compared with an incidence of 6.0/million for acute myeloid leukaemia (AML), and of 0.5/million for chronic myeloid leukaemia. There was a non-significant (P = 0.19) trend toward an increase in MDS incidence with time, the increase was partly explained by an increasing number of patients with Down syndrome. Associated abnormalities were found in 48% of the MDS cases with Down syndrome as the most common (seven cases). Only one third of the MDS cases were correctly registered in the Cancer Registry and less than half of the eligible MDS patients were enrolled on a cooperative group study. Data on MDS from treatment-based studies and cancer registries were inaccurate and seemed to significantly underestimate the incidence of MDS in children.