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Childhood myelodysplastic syndrome in Denmark: incidence and predisposing conditions
H Hasle1, G Kerndrup, B B Jacobsen
1Department of Pediatrics, Odense University Hospital, Denmark.
Abstract:
Myelodysplastic syndrome (MDS) in childhood is considered to be very rare, but sound epidemiologic data are lacking. We report a population-based study of MDS in Denmark from 1980 to 1991. The medical charts were reviewed of 988 children identified from the Danish National Hospital Discharge Registry with a diagnosis of myeloid leukemia or blood cytopenia. Blood and bone marrow smears from all cases of possible MDS were re-evaluated. The cases were categorized according to the FAB classification, with the exception of chronic myelomonocytic leukemia (CMML) in which more than 5% myeloblasts in the blood was accepted. Juvenile chronic myeloid leukemia (JCML) was included as CMML. MDS was diagnosed in 46 children representing 9% of all hematologic malignancies in children less than 15 years of age. The annual incidence was 4.0/million and did not increase with time. Refractory anemia with excess of blasts and CMML each accounted for one third of the cases. Down syndrome was present in seven children. Other predisposing conditions included Fanconi anemia, neurofibromatosis, constitutional trisomy 8 mosaicism, and familial leukemia. Only one child had therapy-related MDS. The study indicates that the incidence of childhood MDS is higher than generally assumed and approximate to the incidence of acute myeloid leukemia.
Insights
Childhood myelodysplastic syndromes (MDS) are more common than previously thought, with an incidence similar to acute myeloid leukemia. This population-based study provides crucial epidemiologic data for this rare pediatric condition.
Area of Science:
- Pediatric Hematology
- Epidemiology
- Oncology
Background:
- Childhood myelodysplastic syndrome (MDS) is considered rare, but robust epidemiologic data are scarce.
- Accurate incidence rates are essential for understanding disease burden and resource allocation.
Purpose of the Study:
- To determine the population-based incidence and characteristics of childhood MDS in Denmark.
- To provide updated epidemiologic data on this rare pediatric hematologic malignancy.
Main Methods:
- Population-based retrospective study in Denmark (1980-1991).
- Review of medical charts for 988 children diagnosed with myeloid leukemia or blood cytopenia.
- Re-evaluation of blood and bone marrow smears using FAB classification criteria.
Main Results:
- MDS was diagnosed in 46 children, representing 9% of pediatric hematologic malignancies.
- The annual incidence was 4.0 per million children, with no temporal increase.
- Refractory anemia with excess blasts and chronic myelomonocytic leukemia (CMML) were the most common subtypes.
Conclusions:
- Childhood MDS incidence is higher than previously assumed, approximating that of acute myeloid leukemia.
- Down syndrome and other genetic conditions are associated with childhood MDS.
- Therapy-related MDS is rare in children.