Abstract

Iron overload can lead to tissue damage derived from free radical toxicity. Phlebotomy is the treatment of choice for treating iron overload. However, iron chelating therapy can be used if phlebotomies are impossible, mainly because of anemia. In thalassemia major, iron chelating therapy has dramatically improved life expectancy; it is also used in sickle cell disease and myelodysplastic syndromes. Desferioxamine is the gold standard of iron chelation, but parenteral administration and the burden of a daily infusion pump hinder optimal compliance. Deferiprone is orally active but should be administered three times a day. It has the advantage of removing toxic iron from myocardium, but agranulocytosis (1 %) can limit its use. Deferasirox is orally active in a single daily dose, is well tolerated but its cardiac effect is limited. Iron chelating therapy can be considered if serum ferritin is above 1000μg/L and if liver iron concentration assessing by MRI exceeds 80μmol/g. MRI is a very important mean to monitor cardiac iron load. If the relaxing parameter T2* is lower than 20ms, a cardiac effective iron chelator agent or an association with deferoxamine should be used. Benefit/risk ratio should be closely evaluated, mainly in myelodysplastic syndromes.

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