Iron overload in thalassaemia intermedia: reassessment of iron chelation strategies. 2009

Ali Taher, and Chaim Hershko, and Maria Domenica Cappellini
Department of Internal Medicine, Haematology-Oncology Division, American University of Beirut, Medical Centre, Beirut, Lebanon. ataher@aub.edu.lb

Thalassaemia intermedia (TI) is a syndrome marked by its diverse underlying genetic basis although its pathophysiology remains unclear, particularly regarding the nature of iron loading and toxicity. It is, however, evident that there are key differences from the extensively studied thalassaemia major (TM) population and caution is required when assessing iron load based on serum ferritin values, as this approach is known to underestimate the true extent of iron loading in patients with TI. Although effective iron chelation therapy has been available for many years, studies in TI-specific populations are rare and evidence suggests that management of iron levels may be less rigorous than in patients with TM and other chronic anaemias. Better understanding of the need to assess and treat iron overload in both transfused and non-transfused TI patients is clearly required.

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
D007502 Iron Chelating Agents Organic chemicals that form two or more coordination links with an iron ion. Once coordination has occurred, the complex formed is called a chelate. The iron-binding porphyrin group of hemoglobin is an example of a metal chelate found in biological systems. Iron Chelates,Agents, Iron Chelating,Chelates, Iron,Chelating Agents, Iron
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D013789 Thalassemia A group of hereditary hemolytic anemias in which there is decreased synthesis of one or more hemoglobin polypeptide chains. There are several genetic types with clinical pictures ranging from barely detectable hematologic abnormality to severe and fatal anemia. 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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