Myelodysplasia

Abstract

Myelodysplasia comprises a group of clonal haematopoietic stem cell disorders characterized by cytopenia and dysplasia in one or more cell lineage(s), and a tendency to evolve to acute myeloid leukaemia. Myelodysplasia is much rarer in children than in adults. This chapter describes the epidemiology, classification, clinical features and management of myelodysplasia in children which differs considerably from that in adults. Juvenile myelomonocytic leukaemia is regarded as a myelodyplastic / myeloproliferative disease and is unique to childhood. Supportive care is important for all children with myelodysplasia. Whilst a few children with low grade myelodysplasia may be managed with observation only, most children with advanced myelodysplasia and juvenile myelomonocytic leukaemia require haemopoietic stem cell transplantation.