title: How we manage iron overload in sickle cell patients.
abstract: Blood transfusion plays a prominent role in the management of patients with sickle cell disease (SCD), but causes significant iron overload. As transfusions are used to treat the severe complications of SCD, it remains difficult to distinguish whether organ damage is a consequence of iron overload or is due to the complications treated by transfusion. Better management has resulted in increased survival, but prolonged exposure to iron puts SCD patients at greater risk for iron-related complications that should be treated. The success of chelation therapy is dominated by patient adherence to prescribed treatment; thus, adjustment of drug regimens to increase adherence to treatment is critical. This review will discuss the current biology of iron homeostasis in patients with SCD and how this informs our clinical approach to treatment. We will present the clinical approach to treatment of iron overload at our centre using serial assessment of organ iron by magnetic resonance imaging.
authors.affiliation: Hematology Section, Children's Centre for Cancer, Blood Diseases and Bone Marrow Transplantation, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA.
authors.forename: Thomas D
authors.initials: TD
authors.lastname: Coates
authors.affiliation: Division of Cardiology at the Children's Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA.
authors.forename: John C
authors.initials: JC
authors.lastname: Wood
mesh_terms: D000755:Anemia, Sickle Cell / Q000150:complications* / Q000628:therapy; D015913:Chelation Therapy / Q000379:methods; D006706:Homeostasis / Q000502:physiology; D006801:Humans; D007501:Iron / Q000009:adverse effects / Q000378:metabolism; D007502:Iron Chelating Agents / Q000627:therapeutic use; D019190:Iron Overload / Q000175:diagnosis / Q000209:etiology / Q000628:therapy*; D008279:Magnetic Resonance Imaging; D055118:Medication Adherence; D065227:Transfusion Reaction
chemical_list: D007502:Iron Chelating Agents; D007501:Iron
keywords: anaemia; chelator; iron overload; management; sickle cell disease