IV. MANAGEMENT
Venesection with removal of 500 ml of blood every 2 weeks for several months followed by monthly venesection for a year usually results in a reduction of serum ferritin level to less than 50 mg/L. Venesections are continued every 3 months or more frequently to maintain a ferritin level 50 mg/L range. Alcohol increases absorption of iron and should be curtailed, especially because cirrhosis is an outcome of the iron overload. If the serum iron is kept within the normal range before serious cardiac involvement has occurred, the prognosis is good. If cardio-myopathy resulting in heart failure has occurred, the prognosis is poor.
Iron chelation may reverse some degree of cardiac dysfunction. Intravenous desferrioxamine on a 24-h per day regimen resulted in some improvement in left ventric¬ular dysfunction and improvement in ejection fraction in some patients with severe cardiac dysfunction caused by transfusional iron overload.
BIBLIOGRAPHY
Andrews, N. C. Disorders of iron metabolism. N. Engl. J. Med., 341:1986–95, 1999.
Andrews, N. C., and Levy, J. E. Iron is hot. An update on the patho-physiology of hemochromatosis. Blood, 92:1845–51, 1998.