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Haemochromatosis
Background
Haemochromatosis (Hemochromatosis - US) is the medical term describing the presence of excess iron in the body. It may be inherited or acquired.
Acquired haemochromatosis usually occurs due to accumulated iron from frequent blood transfusions given to treat haemolytic anaemias. Often these are necessary for children with anaemias, and therefore iron overload can affect this age group. Features include cardiac and liver disease. This type of haemochromatosis is treated, and indeed prevented, by the use of iron chelation therapy, using a drug such as desferrioxamine which binds iron in the body and allows it to be excreted in the urine. As with genetic haemochromatosis, iron removal may not reverse disease, because of the tissue damage that has already occurred.
Genetic haemochromatosis usually has autosomal recessive inheritance and is the result of excessive absorption of iron from the intestine despite normal or high body iron stores. Over ninety per cent of patients are homozygous for the C282Y mutation in the HFE gene on chromosome 6. Iron accumulates in the liver, pancreas, heart, joints and pituitary. Iron overload only reaches levels where there is tissue damage in the third to fifth decade of life, and therefore children are rarely if ever affected. Moreover, in many people with the genetic condition, iron accumulates only slowly and may not cause the symptoms described below.
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