MYELODYSPLASTIC SYNDROMES IN CHILDHOOD - IS THE FAB CLASSIFICATION RELEVANT - REPORT OF 81 CHILDREN FROM A FRENCH MULTICENTER STUDY

We reviewed the peripheral blood and bone marrow smears of 81 children with myelodysplastic syndrome (MDS). The morphological FAB classifica tion was applicable in 59 children (72,8%): RAEB and RAEBt were the mo st frequent, 32 cases (39,5%). CMML was observed in 15 cases (18,5%) a nd in 25% of them, serological evidence for a recent EBV infection was demonstrated. In 22 cases (27,2%), the FAB classification was not con venient. In some of these children, dysmyelopoiesis was associated wit h constitutional disorders. Among these various inherited conditions, Down syndrome in which myelodysplasia is the expression of an abnormal clonal hematopoiesis, and mitochondrial cytopathies in which MDS is t he hematological expression of a polyclonal multi-organ disease. The F AB classification does not appear to be satisfactory for all the disor ders included in the group of childhood MDS and should be modified for specific use in children.