Myelodysplastic syndromes in children: Observations on five cases

Five paediatric patients with a myelodysplastic syndrome (MDS) (age range 4-12 years) are described. The frequency of MDS in our institution in the last 13 years is 1.12% of all leukaemic syndromes. Diagnosis and classification were made according to the recent proposals of the FAB Co-operative Group: four refractory anaemia with excess of blasts in transformation (RAEB-T) and one refractory anaemia with excess of blasts (RAEB). The haematqlogical picture at onset is presented with morphological, cytochemical, immunological and cytogenetic data. Two patients had trisomy 8 and one had monosomy 7. In three patients an evolution towards acute myeloid leukaemia was observed. Multiple drug treatment used in the chronic phase and/or during transformation in acute leukaemia did not produce complete remission. Low-dose Ara-C was employed in one case in the chronic phase without improvement. Four patients died 3-17 months after diagnosis. One is alive with persistent leukaemia 8 months after diagnosis.