Abstract
We present 4 cases of myelodysplastic syndrome (MDS) in children : 1 case of refractory anemia (RA), 1 case of RA with excess of blasts (RAEB) and 2 cases of RAEB in transformation (RAEB-T) at the time of diagnosis. RAEB-T eventually transformed to acute myeloblastic leukemia (AML). One case of the RAEB-T had hypoplastic marrow. Chromosomal analysis of 3 cases showed abnormalities of the 7th and/or 8th chromosomes. Bone marrow progenitor cell assay showed decreased colony-forming units in all cases, but many clusters were observed in 2 cases. The cases of RAEB and RAEB-T were treated with anti-leukemic drugs and complete remission was induced. They relapsed 7-8 months later and died in 14-62 months from the initial diagnosis. One case of RA showed transient improvement of anemia with Ubenimex. In conclusion, further propspective group study of chemotherapy according to the types of MDS should be performed in order to improve the prognosis.
