Abstract
Myelodysplastic syndromes (MDS) in childhood are a heterogenous group of clonal stem cell disorders accounting for less than10%of all hematologic malignancies in childhood. Classification of MDS in childhood has been subject to some controversy. We have argued that the French-American-British (FAB) classification with some modification is the most useful framework for the study of MDS in childhood. In contrast to adulthood, CMML and RAEB are the most common FAB subtypes in childhood. MDS in childhood may arise from a number of predisposing conditions. Hematopoietic stem cell transplantation is generally the treatment of choice, while the role of chemotherapy is less clear. With prospective studies of the European Working-Group of MDS (EWOG-MDS) it is hoped to improve diagnostic procedures and therapy of children and adolescents with MDS.
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