[Myelodysplastic syndrome: a review]

Abstract

Myelodysplastic syndromes (MDS) are heterogeneous diseases with cytopenia in the peripheral blood, dysplasia of two or three cell lines, and a low leukemic blast count in the bone marrow and peripheral blood. The syndrome is rare in childhood (1%-2% of all acute leukemias). In the advanced subtypes of MDS with 5% to 30% of blasts the risk of progression into acute leukemia is high, especially in childhood. Cytogenetic abnormalities like monosomy 7 are typical for children with MDS, and probably are an unfavourable prognostic factor. The overall prognosis is almost the same as in adults, with a median survival time of 19 months. There are no well-established therapy strategies in MDS. Intensive chemotherapy may be beneficial, at least in children and young adults, but the decision when to start treatment is difficult. At present allogeneic bone marrow transplantation is the only curative therapy available for MDS.