A REAPPRAISAL OF HEPATIC SIDEROSIS IN PATIENTS WITH END-STAGE CIRRHOSIS - PRACTICAL IMPLICATIONS FOR THE DIAGNOSIS OF HEMOCHROMATOSIS

The aim of this study was to describe the histologic pattern of iron d istribution in end-stage cirrhosis due to various causes and to test t he reliability of the hepatic iron index (equal to hepatic iron concen tration divided by age) in excluding or confirming associated hemochro matosis in such a condition. Large slices of the resected livers of 30 patients transplanted for alcoholic and/or viral end-stage cirrhosis were assessed histologi cally for iron distribution and biochemically for hepatic iron concentration in the least and the most iron-overload ed nodules of each case. HLA-A3 was used as the marker for the hemochr omatosis gene in the population studied. Intranodular parenchymal side rosis was found in 23 cases (12 spotty, 11 diffuse) with diffuse intra biliary iron deposits apparent in only two cases. Although in 14 patie nts the hepatic iron index was significantly high (>1.9) so as to sugg est hemochromatosis, these cases did not correspond to homozygous hemo chromatosis with respect to the prevalence of HLA-A3 antigen. End-stag e cirrhosis arising from different causes is frequently complicated by parenchymal siderosis that may mimic hemochromatosis, including a hep atic iron index greater than 1.9. The diagnosis of hemochromatosis in patients with end-stage cirrhosis, even those with a hepatic iron inde x greater than 1.9, should rely mainly on clinical and histologic data .