Treatment of Systemic Iron Overload
The long-term consequences of transfusional iron overload, as well as those resulting from Fenetic haemochromatosis, can be effectively prevented by controlling iron overload. Treatment of the latter is usually relatively simple by weekly phlebotomy until body iron levels approach those of healthy individuals. By contrast, the treatment and monitoring of transfusional iron overload is more complex. This entails iron-chelating therapy to bind excess body iron and excrete chelate-complexed iron in faeces and/or urine. Careful titration of chelation dosing is necessary, taking into account current levels of body iron as well as the rates of iron accumulation from blood transfusion. Failure to adjust the chelation regime for these variables can result in either serious iron toxicity to heart, endocrine system and liver, or conversely in toxicities resulting from over-chelation. Appropriate adjustments necessitate careful monitoring of total body iron, estimated from liver iron concentration (LIC), as well as assessment of iron distribution outside the liver, particularly heart and endocrine system using MRI techniques. This article will describe how licensed iron chelators, namely deferiprone, desferrioxamine and deferasirox can be used alone or in combination to control transfusional iron overload or its unwanted effects. Practical approaches to monitoring the levels and distribution of iron overload, as well as unwanted effects of excess iron or excess iron chelation are also discussed.