title: Clinical relevance of anemia and transfusion iron overload in myelodysplastic syndromes.
abstract: Most patients with myelodysplastic syndrome eventually become dependent on regular red cell transfusions. This dependency has a negative impact on clinical outcome, primarily because it may be associated with more severe marrow failure. In addition, however, transfusion dependency may involve clinical consequences of chronic anemia and iron overload. Although transfusion iron is primarily taken up by the reticuloendothelial cells, the metal is later redistributed to parenchymal cells. This redistribution is modulated by several factors, including the degree of ineffective erythropoiesis through its suppressive effect on hepcidin production. Body iron status is routinely assessed by serum ferritin and transferrin saturation, but there is a need of reliable tools for locating iron accumulation in patients. Magnetic resonance imaging T2* provides a non-invasive method for detecting and quantifying both liver and myocardial iron overload. Clinical consequences of parenchymal iron overload have been reported not only in thalassemia major, but also in patients with myelodysplastic syndrome. Transfusion-dependent patients with isolated erythroid dysplasia and low risk of leukemic evolution are more likely to develop parenchymal iron overload and its toxicity, and therefore may benefit from chelation therapy. There may also be a benefit of chelation therapy in patients with transfusion iron overload undergoing allogeneic stem cell transplantation. Deferoxamine and deferasirox are currently available for treatment of transfusion iron overload in patients with myelodysplastic syndrome.
authors.affiliation: Department of Hematology Oncology, University of Pavia & Fondazione IRCCS Policlinico San Matteo, Pavia, Italy. mario.cazzola@unipv.it
authors.forename: Mario
authors.initials: M
authors.lastname: Cazzola
authors.forename: Matteo G
authors.initials: MG
authors.lastname: Della Porta
authors.forename: Luca
authors.initials: L
authors.lastname: Malcovati
mesh_terms: D000740:Anemia / Q000453:epidemiology* / Q000503:physiopathology; D023181:Antimicrobial Cationic Peptides / Q000096:biosynthesis; D001854:Bone Marrow Cells / Q000502:physiology; D004905:Erythrocyte Aging / Q000502:physiology; D017707:Erythrocyte Transfusion / Q000009:adverse effects*; D004920:Erythropoiesis / Q000502:physiology; D064451:Hepcidins; D006801:Humans; D007408:Intestinal Absorption; D007501:Iron / Q000378:metabolism*; D019190:Iron Overload / Q000175:diagnosis / Q000209:etiology*; D008264:Macrophages / Q000502:physiology; D008279:Magnetic Resonance Imaging; D012157:Mononuclear Phagocyte System / Q000502:physiology; D009190:Myelodysplastic Syndromes / Q000097:blood* / Q000150:complications* / Q000628:therapy
chemical_list: D023181:Antimicrobial Cationic Peptides; C578702:HAMP protein, human; D064451:Hepcidins; D007501:Iron