Management

• Iron chelation therapy by parenteral desferrioxamine is the only treatment for patients with transfusion haemosiderosis, who remain anaemic. Haemosiderosis due to previous transfusions in conditions where Hb now normal e.g. treated AML, may be venesected to remove iron.

• Regular treatment is required in transfusion dependent children if they are to avoid the consequences of iron overload in the second decade of life.

• SC administration of desferrioxamine by portable syringe pump over 9-12h on 5-7 nights/week is a common regimen.

• Ascorbic acid supplementation may help mobilise iron and increase excretion with desferrioxamine but can cause hazardous redistribution of storage iron.

• Early and regular desferrioxamine infusion 5 hepatic iron and improves hepatic function, promotes growth and sexual development and protects against heart disease and early death.

• Much work has been expended in the search for an effective non-toxic oral chelator and deferiprone is currently under evaluation in clinical trials. 131

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