Cardiac iron across different transfusion-dependent diseases.
Iron overload occurs in patients who require regular blood transfusions to correct genetic and acquired anaemias, such as beta-thalassaemia major, sickle cell disease, and myelodysplastic syndromes. Although iron overload causes damage in many organs, accumulation of cardiac iron is a leading cause of death in transfused patients with beta-thalassaemia major. The symptoms of cardiac iron overload will occur long after the first cardiac iron accumulation, at a point when treatment is more complex than primary prevention would have been. Direct measurement of cardiac iron using T2* magnetic resonance imaging, rather than indirect methods such as measuring serum ferritin levels or liver iron concentration have contributed to earlier recognition of myocardial iron loading and prevention of cardiac toxicity. Cardiac siderosis occurs in all transfusional anaemias, but the relative risk depends upon the underlying disease state, transfusional load, and chelation history. All three available iron chelators can be used to remove cardiac iron, but each has unique physical properties that influence their cardiac efficacy. More prospective trials are needed to assess the effects of single-agent or combination iron chelation therapy on the levels of cardiac iron and cardiac function. Ultimately, iron chelation therapies should be tailored to meet individual patient needs and lifestyle demands.
Wood
John C
JC
Division of Pediatric Cardiology, Children's Hospital Los Angeles and Keck School of Medicine, University of Southern California, Los Angeles, CA 90027, USA. jwood@chla.usc.edu
D000755:Anemia, Sickle Cell / Q000150:complications; D006801:Humans; D007502:Iron Chelating Agents / Q000627:therapeutic use; D019190:Iron Overload / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D009190:Myelodysplastic Syndromes / Q000150:complications; D009206:Myocardium / Q000378:metabolism*; D065227:Transfusion Reaction; D017086:beta-Thalassemia / Q000150:complications
D007502:Iron Chelating Agents