title: The pathophysiology of transfusional iron overload.
abstract: The pathophysiologic consequences of transfusional iron overload (TIO) as well as the benefits of iron chelation therapy are best described in thalassemia major, although TIO is increasingly seen in other clinical settings. These consequences broadly reflect the levels and distribution of excess storage iron in the heart, endocrine tissues, and liver. TIO also increases the risk of infection, due to increased availability of labile iron to microorganisms. The authors suggest that extrahepatic iron distribution, and hence toxicity, is influenced by balance between generation of nontransferrin-bound iron from red cell catabolism and the utilization of transferrin iron by the erythron.
authors: Department of Haematology, University College London, 72 Huntley Street, London WC1E 6BT, UK. Electronic address: j.porter@ucl.ac.uk.
authors: John B
authors: JB
authors: Porter
authors: Department of Haematology, University College London, 72 Huntley Street, London WC1E 6BT, UK.
authors: Maciej
authors: M
authors: Garbowski
mesh_terms: D000755:Anemia, Sickle Cell / Q000150:complications / Q000628:therapy; D006801:Humans; D007501:Iron / Q000378:metabolism; D019190:Iron Overload / Q000209:etiology* / Q000378:metabolism; D065227:Transfusion Reaction; D017086:beta-Thalassemia / Q000150:complications / Q000628:therapy
chemical_list: D007501:Iron
keywords: Blood transfusion; Extra-hepatic iron distribution; Iron overload; Mechanism; NTBI; Pathophysiology; Sickle cell disease; Thalassemia