Journal
BLOOD REVIEWS
Volume 22, Issue -, Pages S29-S34Publisher
CHURCHILL LIVINGSTONE
DOI: 10.1016/S0268-960X(08)70006-7
Keywords
Cardiac dysfunction; Cardiac iron overload; Iron chelation therapy; Myelodysplastic syndromes; Transfusion therapy
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Many patients with myelodysplastic syndromes (MDS) have severe anaemia. However, regular blood transfusions, which are widely used to maintain quality of life and prevent anaemia-retated morbidity and mortality, have a negative impact on survival as a result of iron overload. Retrospective surveys have shown an association of transfusion dependence with hepatic, pituitary, and pancreatic dysfunction, cardiac failure, and cardiac death. Survival is significantly decreased in transfusion-dependent patients, and the main cause of non-leukaemic death is cardiac failure. However, iron chelation therapy reduces serum ferritin levels and is associated with significantly improved survival in patients with MDS. Current guidelines recommend starting iron chelation therapy after 25-50 units of blood have been transfused, or when serum ferritin levels rise above 1,000-2,000 mu g/L. The patients who are most likely to benefit from iron chelation therapy are those who have low-risk disease (International Prognostic Scoring System tow or intermediate-1 risk) with a life expectancy of more than 1 year. More specific studies in patients with MDS are needed to evaluate the impact of iron chelation therapy on morbidity and mortality, and provide a stronger evidence base for treatment guidelines. (C) 2008 Published by Elsevier Ltd.
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