Transfusion hemosiderosis in the context of "Chelation therapy"

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⭐ Core Definition: Transfusion hemosiderosis

Transfusional hemosiderosis is the accumulation of iron in the body due to frequent blood transfusions. Iron accumulates in the liver and heart, but also endocrine organs. Frequent blood transfusions may be given to many patients, such as those with thalassemia, sickle cell disease, leukemia, aplastic anemia, or myelodysplastic syndrome, among others. It is diagnosed with a blood transferrin test and a liver biopsy. It is treated with venipuncture, erythrocytapheresis, and iron chelation therapy.

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Transfusion hemosiderosis in the context of Hemochromatosis

Iron overload is the abnormal and increased accumulation of total iron in the body, leading to organ damage. The primary mechanism of organ damage is oxidative stress, as elevated intracellular iron levels increase free radical formation via the Fenton reaction. Iron overload is often primary (i.e, hereditary haemochromatosis, aceruloplasminemia) but may also be secondary to other causes (i.e., transfusional iron overload). Iron deposition most commonly occurs in the liver, pancreas, skin, heart, and joints.

People with iron overload classically present with the triad of liver cirrhosis, secondary diabetes mellitus, and bronze skin. However, due to earlier detection nowadays, symptoms are often limited to general chronic malaise, arthralgia, and hepatomegaly.

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