Recent advances in improving the management of sickle cell disease

Abstract 

Appropriate blood transfusions can both prevent and treat serious complications related to sickle cell disease (SCD), but inevitably lead to iron overload and its complications in terms of morbidity and mortality. Transfusion history and serial serum ferritin level assessments are convenient and cost-effective measures for monitoring iron overload in SCD. Monitoring and treatment of patients with SCD-related iron overload lags behind the standards of care for b-thalassaemia. Data from the EPIC trial suggest suboptimal iron chelation in SCD, highlighting the need to carefully monitor iron levels and initiate iron chelation therapy to avoid serious clinical sequelae. Deferasirox is effective and well tolerated in patients with SCD, including paediatric patients. Patient compliance is, however, important for effectiveness of iron chelation therapy.

Keywords:  Sickle cell disease , Iron overload , Stroke , Iron chelation therapy , Deferasirox

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