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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.
The human body has protective mechanisms in place to prevent excess free ferric iron from circulating the body. When being transported throughout the body, iron is bound to an iron transporting protein called transferrin to prevent iron from being absorbed into different cells. [6] Any excess iron is stored as ferritin in the liver. [6]
Serum ferritin is a low cost, readily available, and minimally invasive method for assessing body iron stores. However, the major problem with using it as an indicator of hemosiderosis is that it can be elevated in a range of other medical conditions unrelated to iron levels including infection, inflammation, fever, liver disease, renal disease ...
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Hemosiderin or haemosiderin is an iron-storage complex that is composed of partially digested ferritin and lysosomes. The breakdown of heme gives rise to biliverdin and iron. [1] [2] The body then traps the released iron and stores it as hemosiderin in tissues. [3] Hemosiderin is also generated from the abnormal metabolic pathway of ferritin. [3]
Transfusion hemosiderosis can cause permanent damage to tissues that may lead to death. [2] Tissue damage can remain even after chelation therapy. [2] Outcomes are usually worse in patients who require blood transfusions compared to those who can have alternative therapies. [2] Cardiomyopathy and cardiac arrhythmia are often a cause of death. [1]
Arrhythmia or abnormal heart rhythm can cause heart palpitations, chest pain, and light-headedness, and is occasionally life-threatening. This condition can often be reversed with treatment. [citation needed] The pancreas, which also stores iron, is very important in the body's mechanisms for sugar metabolism.
Internal bleeding (also called internal haemorrhage) is a loss of blood from a blood vessel that collects inside the body, and is not usually visible from the outside. [1] It can be a serious medical emergency but the extent of severity depends on bleeding rate and location of the bleeding (e.g. head, torso, extremities).