Abstract 17

A retrospective analysis of the clinical and laboratory features of children with myelodysplastic syndromes (MDS) was undertaken from articles which described patients who had been classified within the French American British grouping, (FAB). This study also included children with juvenile chronic myelomonocytic leukemia (JCML) and monosomy 7 (Mo7). 340 patients were described in 27 publications. The mean presentation age was 5.91 (SD 5.04) years and 34.9% were female. Constitutional alterations were described in 68 where the earlier forms of MDS, refractory anemia (RA) and RA with excessive blasts predominated, and were associated with a significantly longer survival. Progression of the diagnosis was noted in 61. Cytogenetic anomalies were detected in 59% of 227 patients and in 67 it was to Mo7. Among those with Mo7, clinical and laboratory characteristics as well as survival, closely followed their FAB type. Of the treatment options described, survival was significantly higher in those who underwent bone marrow transplant (BMT) (46.9%; p=0.00021). Among children with JMML, time to death of patients not receiving a BMT was shortest in those displaying features of JCML (absence of constitutional derangement, thrombocytopenia and elevated Hb F). We conclude that CMML and JCML may have biological differences, that Mo7 disorders are clinically and morphologivally heterogeneous and should not be grouped into a single entity and finally, that BMT remains the treatment of choice, as for those with primary MDS, intensive chemotherapy is not better than supportive measures.