Myelodysplastic syndromes

Myelodysplasia is one of the Cinderella subjects of paediatric haematology and oncology. Although cases are rare and probably do not account for more than 3% of haematological malignancies, patients presenting with these disorders pose a very serious challenge to successful diagnosis and management with which we are only just coming to grips. In fact, such is the previous state of confusion that we have no idea of the prevalence of the component disorders and there is little doubt that under diagnosis was itself prevalent. The first tentative steps have now been taken out of the darkness and probable cures are being achieved in previously almost uniformly fatal conditions. The first moves in the right direction were achieved by abandoning the paediatricians inherent love of syndromes and concentrating, with our adult haematology colleagues, on getting some sense and science into classification of the disorders. tric literature, juvenile chronic myeloid leukaemia (JCML) and infantile monosomy 7 syndrome. Both of these disorders will presumably take their place under more appropriate headings when more systematic studies are completed, for example, JCML shows some features akin to a well described adult counterpart, chronic myelomonocytic leukaemia (CMML). Table 2 shows a recommended list of investigations required for the diagnosis and classification of myelodysplasia. I would like to expend a little space on the specific features of the disorders listed in table 1. However, there is insufficient space to deal adequately with chronic myeloid leukaemia, which has been extensively reviewed elsewhere.5-10 Essential thrombocythaemia and polycythaemia are excessively rare in childhood and the few authenticated cases have also been reviewed elsewhere. 113 Malignant myelofibrosis as seen in adults'4 1l is not seen in children and modern techniques have shown