Management of hemochromatosis

The complications of iron overload in hemochromatosis can be avoided by ear ly diagnosis and appropriate management. Therapeutic phlebotomy is used to remove excess iron and maintain low normal body iron stores, and it should be initiated in men with serum ferritin levels of 300 mu g/L or more and in women with serum ferritin levels of 200 mu g/L or more, regardless of the presence or absence of symptoms. Typically, therapeutic phlebotomy consists of 1) removal of 1 unit (450 to 500 mL) of blood weekly until the serum fe rritin level is 10 to 20 mu g/L and 2) maintenance of the serum ferritin le vel at 50 mu g/L or less thereafter by periodic removal of blood. Hyperferr itinemia attributable to iron overload is resolved by therapeutic phlebotom y. When applied before iron overload becomes severe, this treatment also pr events complications of iron overload, including hepatic cirrhosis, primary liver cancer, diabetes mellitus, hypogonadotrophic hypogonadism, joint dis ease, and cardiomyopathy. In patients with established iron overload diseas e, weakness, fatigue, increased hepatic enzyme concentrations, right upper quadrant pain, and hyperpigmentation are often substantially alleviated by therapeutic phlebotomy. Patients with liver disease, joint disease, diabete s mellitus and other endocrinopathic abnormalities, and cardiac abnormaliti es often require additional, specific management. Dietary management of hem ochromatosis includes avoidance of medicinal iron, mineral supplements, exc ess vitamin C, and uncooked seafoods. This can reduce the rate of iron reac cumulation; reduce retention of nonferrous metals; and help reduce complica tions of liver disease, diabetes mellitus, and Vibrio infection. This compr ehensive approach to the management of hemochromatosis can decrease the fre quency and severity of iron overload, improve quality of life, and increase longevity.