Childhood myelodysplastic syndromes in a Brazilian population

Fourteen pediatric cases of myelodysplastic syndrome according to French-Am erican-British Cooperative Group (FAB) criteria were identified in a retros pective review of all cases of hematologic malignancies referred to the Ped iatric Oncology division of the Cancer Hospital A. C. Camargo over a 12-yea r period: 1 case of refractory anemia, 8 cases of refractory anemia with ex cess of blasts, and 1 case of refractory anemia with excess of blasts in tr ansformation. Three children had features consistent with chronic myelomono cytic leukemia, and one child was diagnosed with secondary myelodysplastic syndrome. The median age was 3.5 years (1 month-11 years). In 11/14 casus t he disease evolved to acute leukemia. In 3 patients blasts had morphologica l and cytochemical features of lymphoblasts. Two of these patients had a go od response to acute lymphoblastic leukemia chemotherapy protocol. The time of progression to leukemia in these cases was shorter than in those who ev olved to acute myeloid leukemia. The authors believe this to be the first s eries of pediatric myelodysplastic syndrome documented in Brazil. Cases wer e characterized by aggressive FAB type, conspicuous cell atypias in all 3 h emopioetic cell fines, and a high rate of evolution to acute leukemia.