Haemat Onco » Miscellaneous

Haemochromatosis

Haemochromatosis is characterized by a progressive increase in total body iron stores with abnormal iron deposition in multiple organs. Primary haemochromatosis is a genetic disorder, whereas secondary haemochromatosis can be the result of a variety of disorders, most commonly chronic haemolytic anaemias. Clinical manifestations include liver disease, skin pigmentation, diabetes mellitus, arthropathy, Impotence in males and cardiac enlargement, with or without heart failure or conduction defects Haemochromatosis is suggested by a persistently elevated transferrin saturation in the absence of other causes of iron overload. MRI is the best imaging examination to evaluate abnormal iron deposition in the liver. The goal of therapy in patients with iron overload disorders is to remove the iron before it can produce irreversible parenchymal damage.

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