Childhood Myelodysplastic Syndromes In A Brazilian Population

Fourteen pediatric cases of myelodysplastic syndrome according to French-American-British Cooperative Group (FAB) criteria were identified in a retrospective review of all cases of hematologic malignancies referred to the Pediatric Oncology division of the Cancer Hospital A. C. Camargo over a 12- year period: 1 case of refractory anemia, 8 cases of refractory anemia with excess of blasts, and 1 case of refractory anemia with excess of blasts in transformation. Three children had features consistent with chronic myelomonocytic leukemia, and one child was diagnosed with secondary myelodysplastic syndrome. The median age was 3.5 years (1 month-11 years). In 11/14 cases the disease evolved to acute leukemia. In 3 patients blasts had morphological and cytochemical features of lymphoblasts. Two of these patients had a good response to acute lymphoblastic leukemia chemotherapy protocol. The time of progression to leukemia in these cases was shorter than in those who evolved to acute myeloid leukemia. The authors believe this to be the first series of pediatric myelodysplastic syndrome documented in Brazil. Cases were characterized by aggressive FAB type, conspicuous cell atypias in all 3 hemopioetic cell lines, and a high rate of evolution to acute leukemia.164347353Brandwein, J.M., Horsman, E.E., Eaves, A.C., Childhood myelodysplasia: Suggested classification as myclodysplastic syndromes based on laboratory and clinical findings (1990) Am J Pediatr Hematol Oncol., 12, pp. 63-70Creutzig, U., Cantu-Rajnoldi, A., Ritter, J., Myelodysplastic syndromes in childhood: Report of 21 patients from Italy and West Germany (1987) Am J Pediatr Hematol Oncol., 9, pp. 324-330Mufti, G.J., Stevens, J.R., Oscier, D.G., Myelodysplastic syndromes: A scoring system with prognostic significance (1985) Br J Hematol., 59, pp. 425-433Bennett, J.M., Catovsky, D., Daniel, M.T., Proposals for the classification of the myelodysplastic syndromes (1982) Br J Haematol., 51, pp. 189-199Hasle, H., Kerndrup, G., Jacobsen, B.B., Childhood myelodysplastic syndrome in Denmark: Incidence and predisposing conditions (1995) Leukemia, 9, pp. 1569-1572Gadner, H., Haas, O.A., Experience in pediatric myelodysplastic syndromes (1992) Hematol Oncol Clin North Am., 6, pp. 655-672Blank, J., Lange, B., Preleukemia in children (1981) J Pediatr., 98, pp. 565-568Bader-Meunier, B., Mielot, F., Tchernia, G., Myelodysplastic syndrome in childhood: Report of 49 patients from a French multicentre study (1996) Br J Hematol., 92, pp. 344-350Hasle, H., Myelodysplastic syndromes in children: Classification, epidemiology and treatment (1994) Leuk Lymphoma., 13, pp. 11-26Luna-Fineman, S., Shannon, K.M., Lange, B.J., Childhood monosomy 7: Epidemiology, biology and mechanistic implication (1995) Blood, 85, pp. 1985-1999Morel, P., Hebbar, M., Lai, J.L., Cytogenetic analysis has strong independent prognostic value in de novo myelodysplastic syndromes and can be incorporated in a new scoring system: A report on 408 cases (1993) Leukemia, 7, p. 1315Hasle, H., Arico, M., Basso, G., Monosomy 7 in myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML) (1996) Med Pediatr Oncol., 27, p. 230. , SIOP Meeting abstract O 78Niemeyer, C., Arico, M., Biond, A., Hematological features in children with chronic myelomonocytic leukemia (CMML) according to karyotype (1996) Med Pediatr Oncol., 27, p. 230. , SIOP Meeting abstract O 77Passmore, S.J., Hann, I.M., Stiller, C.A., Pediatric myelodysplasia: A study of 68 children and a new prognostic scoring system (1995) Blood, 85, pp. 1742-1750Yamamoto, M., Nakagawa, M., Ichimura, N., Lymphoblastic transformation of chronic myelomonocytic leukemia in an infant (1996) Am J Hematol., 52, pp. 212-214Kouides, P.A., Bennett, J.M., Transformation of chronic myelomonocytic leukemia to acute lymphoblastic leukemia: Case report and review of the literature of lymphoblastic transformation of myelodysplastic syndrome (1995) Am J Hematol., 49, pp. 157-162San Miguel, J.F., Hernandez, J.M., Gonzalez-Sarmiento, R., Acute leukemia after a primary myelodysplastic syndrome: Immunophenotypic, genotypic, and clinical characteristics (1991) Blood, 78, pp. 768-774Hamblin, T., Immunologic abnormalities in myelodysplastic syndromes (1992) Hematol Oncol Clin North Am., pp. 571-586. , Koeffler HP, ed. Philadelphia: SaundersNeame, P.B., Soamboonsrup, P., Browman, G., Simultaneous or sequential expression of lymphoid and myeloid phenotypes in acute leukemia (1985) Blood, 65, pp. 142-148Barton, J.C., Conrad, M.E., Parmley, R.T., Acute lymphoblastic leukemia in idiopathic refractory sideroblastic anemia: Evidence for a common lymphoid and myeloid progenitor cell (1980) Am J Hematol., 9, pp. 109-11