The pathophysiology of transfusional iron overload. 2014

John B Porter, and Maciej Garbowski
Department of Haematology, University College London, 72 Huntley Street, London WC1E 6BT, UK. Electronic address: j.porter@ucl.ac.uk.

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.

UI MeSH Term Description Entries
D007501 Iron A metallic element with atomic symbol Fe, atomic number 26, and atomic weight 55.85. It is an essential constituent of HEMOGLOBINS; CYTOCHROMES; and IRON-BINDING PROTEINS. It plays a role in cellular redox reactions and in the transport of OXYGEN. Iron-56,Iron 56
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000755 Anemia, Sickle Cell A disease characterized by chronic hemolytic anemia, episodic painful crises, and pathologic involvement of many organs. It is the clinical expression of homozygosity for hemoglobin S. Hemoglobin S Disease,HbS Disease,Sickle Cell Anemia,Sickle Cell Disease,Sickle Cell Disorders,Sickling Disorder Due to Hemoglobin S,Anemias, Sickle Cell,Cell Disease, Sickle,Cell Diseases, Sickle,Cell Disorder, Sickle,Cell Disorders, Sickle,Disease, Hemoglobin S,Hemoglobin S Diseases,Sickle Cell Anemias,Sickle Cell Diseases,Sickle Cell Disorder
D017086 beta-Thalassemia A disorder characterized by reduced synthesis of the beta chains of hemoglobin. There is retardation of hemoglobin A synthesis in the heterozygous form (thalassemia minor), which is asymptomatic, while in the homozygous form (thalassemia major, Cooley's anemia, Mediterranean anemia, erythroblastic anemia), which can result in severe complications and even death, hemoglobin A synthesis is absent. Anemia, Cooley's,Anemia, Erythroblastic,Anemia, Mediterranean,Hemoglobin F Disease,Thalassemia Major,Thalassemia Minor,Erythroblastic Anemia,Mediterranean Anemia,Microcytemia, beta Type,Thalassemia Intermedia,Thalassemia Major (beta-Thalassemia Major),Thalassemia Minor (beta-Thalassemia Minor),Thalassemia, beta Type,beta Thalassemia,Anemia, Cooley,Anemia, Cooleys,Anemias, Erythroblastic,Anemias, Mediterranean,Cooley's Anemia,Disease, Hemoglobin F,Intermedia, Thalassemia,Intermedias, Thalassemia,Major, Thalassemia (beta-Thalassemia Major),Majors, Thalassemia (beta-Thalassemia Major),Mediterranean Anemias,Microcytemias, beta Type,Minor, Thalassemia (beta-Thalassemia Minor),Minors, Thalassemia (beta-Thalassemia Minor),Thalassemia Intermedias,Thalassemia Major (beta Thalassemia Major),Thalassemia Majors (beta-Thalassemia Major),Thalassemia Minor (beta Thalassemia Minor),Thalassemia Minors (beta-Thalassemia Minor),Thalassemia, beta,Thalassemias, beta,Thalassemias, beta Type,Type Microcytemia, beta,Type Microcytemias, beta,Type Thalassemia, beta,Type Thalassemias, beta,beta Thalassemias,beta Type Microcytemia,beta Type Microcytemias,beta Type Thalassemia,beta Type Thalassemias
D019190 Iron Overload An excessive accumulation of iron in the body due to a greater than normal absorption of iron from the gastrointestinal tract or from parenteral injection. This may arise from idiopathic hemochromatosis, excessive iron intake, chronic alcoholism, certain types of refractory anemia, or transfusional hemosiderosis. (From Churchill's Illustrated Medical Dictionary, 1989) Overload, Iron
D065227 Transfusion Reaction Complications of BLOOD TRANSFUSION. Included adverse reactions are common allergic and febrile reactions; hemolytic (delayed and acute) reactions; and other non-hemolytic adverse reactions such as infections and adverse immune reactions related to immunocompatibility. Delayed Hemolytic Transfusion Reaction,Acute Hemolytic Transfusion Reaction,Blood Transfusion-Associated Adverse Reactions,Delayed Serologic Transfusion Reaction,Febrile Non-Hemolytic Transfusion Reaction,Hemolytic Transfusion Reaction,Hypotensive Transfusion Reaction,Post-Transfusion Purpura,Posttransfusion Purpura,TAGHD,Transfusion-Associated Allergic Reaction,Transfusion-Associated Circulatory Overload,Transfusion-Associated Dyspnea,Transfusion-Associated Graft Vs. Host Disease,Transfusion-Transmitted Infection,Allergic Reaction, Transfusion-Associated,Blood Transfusion Associated Adverse Reactions,Circulatory Overload, Transfusion-Associated,Circulatory Overloads, Transfusion-Associated,Dyspnea, Transfusion-Associated,Febrile Non Hemolytic Transfusion Reaction,Hemolytic Transfusion Reactions,Infection, Transfusion-Transmitted,Post Transfusion Purpura,Posttransfusion Purpuras,Purpura, Post-Transfusion,Purpura, Posttransfusion,Reaction, Hemolytic Transfusion,Reaction, Hypotensive Transfusion,Reactions, Hemolytic Transfusion,Transfusion Associated Allergic Reaction,Transfusion Associated Circulatory Overload,Transfusion Associated Dyspnea,Transfusion Associated Graft Vs. Host Disease,Transfusion Reaction, Hemolytic,Transfusion Reaction, Hypotensive,Transfusion Reactions,Transfusion Reactions, Hemolytic,Transfusion Reactions, Hypotensive,Transfusion Transmitted Infection,Transfusion-Associated Circulatory Overloads,Transfusion-Transmitted Infections

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