Abstract
Myelodysplastic syndrome (MDS) is a set of clonal bone marrow diseases in children that are characterised by peripheral cytopenia, dysplastic alterations in the bone marrow, and inefficient hematopoiesis. MDS is uncommon in children, with just 1-4 occurrences per million children afflicted. Adults, particularly the elderly, are more susceptible to the disease. Some hereditary disorders, such as Fanconi's anaemia, Shwachman's, and Down's syndromes, are known to predispose children to developing MDS. JCML and monosomy 7 syndrome are the two most frequent paediatric MDS types, both of which affect children in their early years. Approximately 20% of juvenile myelodysplastic syndrome (MDS) cases are discovered by chance during normal laboratory testing or during the course of a suspected hereditary bone marrow failure (IBMF). Differentiating MDS with low blast numbers from aplastic anaemia (AA) and MDS with excess blasts from AML are the two key diagnostic issues in this condition. Bone marrow transplantation and stem cell transplantation is the treatment of choice in most cases. In this article we discuss the disease epidemiology, diagnosis, and treatment.