Myelodysplastic Syndromes In Childhood: Is the FAB Classification Relevant? Report of 81 Children From a French Multicentre Study

Abstract

We reviewed the peripheral blood and bone marrow smears of 81 children with myelodysplastic syndrome (MDS). The morphological FAB classification was applicable in 59 children (72,8%): RAEB and RAEBt were the most frequent, 32 cases (39,5%). CMML was observed in 15 cases (18,5%) and in 25% of them, serological evidence for a recent EBV infection was demonstrated. In 22 cases (27,2%), the FAB classification was not convenient. In some of these children, dysmyelopoiesis was associated with 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 the hematological expression of a polyclonal multi-organ disease. The FAB classification does not appear to be satisfactory for all the disorders included in the group of childhood MDS and should be modified for specific use in children.

Key Words:

• Myelodysplasia
• childhood
• FAB classification
• inherited disorders