Haema 2012; 3(2): 143-150
by Maria Psillaki, Katerina Pirovolaki, Helen A. Papadaki
Hematology Clinic, University Hospital, Heraklion, Crete
Abstract
Myelodysplastic syndromes (MDS) are clonal stem cell disorders characterized by ineffective bone marrow hematopoiesis, peripheral blood cytopenias and an increased risk of transformation to acute myeloid leukemia (AML). Treatment of MDS has evolved to encompass a broad spectrum of therapies aiming to inhibit apoptosis, promote hematopoiesis, reduce proliferation of clonal immature cells and inhibit evolution to AML. Appropriate treatment of cytopenias, as well as of other related complications, not only improves quality of life but also may positively influence the overall survival of the patients. Anemia is the most common cytopenia in MDS and the requirements for regular transfusions is a major clinical problems for patients with low risk MDS. An important therapeutic goal in this patient group is to maintain acceptable hemoglobin levels without transfusions. Despite the introduction of novel agents such as lenalidomide and hypomethylating drugs including azacytidine, treatment with erythropoiesis stimulating agents remains the gold standard. For non-responding patients or for those who lose their therapeutic response, chronic transfusion therapy, with or without the addition of chelating agents, is the only option.