LONG-TERM SURVIVAL IN PATIENTS WITH HEREDITARY HEMOCHROMATOSIS

Background & Aims: The course of hereditary hemochromatosis may depend on the degree of iron overload and the time of therapeutic interventi on. This analysis evaluates the impact of early diagnosis and iron rem oval on survival and complications in hereditary hemochromatosis. Meth ods: A cohort of 251 patients with hemochromatosis was followed up for 14.1 +/- 6.8 years, Results: Survival was reduced in the total group of patients when compared with a matched normal population. Survival i n noncirrhotic and nondiabetic patients and in patients diagnosed betw een 1982 and 1991 was identical with rates expected. Survival was redu ced in patients with severe iron overload us. those with less severe o verload. The percentage of early diagnoses increased threefold between 1947 and 1969 to that between 1970 and 1981; there was only a further 20%-25% increase in the last decade. Deaths caused by liver cancer, c ardiomyopathy, liver cirrhosis, and diabetes mellitus were increased a s compared with expected rates. Liver cancers were associated with cir rhosis and amount of mobilizable iron but not with hepatitis B or C ma rkers, Conclusions: Prognosis of hemochromatosis and most of its compl ications, including liver cancer, depend on the amount and duration of iron excess. Early diagnosis and therapy largely prevent the adverse consequences of iron overload.