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Iron overload (also known as haemochromatosis or hemochromatosis) is the abnormal and increased accumulation of total iron in the body, leading to organ damage. [1] The primary mechanism of organ damage is oxidative stress, as elevated intracellular iron levels increase free radical formation via the Fenton reaction.
Normally, HFE facilitates the binding of transferrin, which is iron's carrier protein in the blood. Transferrin levels are typically elevated at times of iron depletion (low ferritin stimulates the release of transferrin from the liver). When transferrin is high, HFE works to increase the intestinal release of iron into the blood.
This iron unavailability potentially leads to mild anemia in type 4A hemochromatosis patients because iron is necessary for hemoglobin synthesis, and red blood cells have a relatively high turnover rate. [4] Over time, iron stores increase, and individuals with type 4A hemochromatosis may develop hepatic fibrosis. [3]
Haemochromatosis is more severe than previously thought and is often diagnosed too late. But the treatment is simple.
Ferrous iron is then absorbed in the small intestine where it is oxidized into its ferric iron (Fe 3+) form before being released into the bloodstream. [4] Free iron in the blood is toxic to the body as it disrupts normal cell function, damaging organs such as the liver, stomach, and cardiovascular system. [4]
Macrophages of the reticuloendothelial system store iron as part of the process of breaking down and processing hemoglobin from engulfed red blood cells. Iron is also stored as a pigment called hemosiderin, which is an ill-defined deposit of protein and iron, created by macrophages where excess iron is present, either locally or systemically, e ...
Elevated levels of methemoglobin in the blood are caused when the mechanisms that defend against oxidative stress within the red blood cell are overwhelmed and the oxygen carrying ferrous ion (Fe 2+) of the heme group of the hemoglobin molecule is oxidized to the ferric state (Fe 3+). This converts hemoglobin to methemoglobin, resulting in a ...
Both these phenomena occur in thalassaemias, with blood transfusion therapy being the major cause of iron overload in thalassaemia major and increased GI absorption being more important in patients with intermedia thalassaemia who are not frequently transfused. Each unit of blood contains about 200 mg iron.