Myelodysplastic syndromes (MDS) in children constitute a heterogeneous
disorder, including 'primary' MDS and MDS associated with constitutio
nal abnormalities. The Franco American-British (FAB) cytological class
ification for adults can be applied for childhood in 50 to 100% of the
cases. The transformation into acute myeloblastic leukemia often occu
rs, but stabilisation or spontaneous regression of the disease may als
o be observed. The therapeutic decision is difficult because there is
no predictive factor of the course of the disease. Allogenic bone marr
ow transplantation is the best curative option when treatment is neces
sary.