Intrahepatocytic Copper and Copper-Binding Protein in Primary Biliary Cirrhosis

Liver tissue samples from 111 patients with various hepatic disorders including 37 with primary biliary cirrhosis (PBC) and 22 with chronic active hepatitis (CAH) were studied. Of the PBC patients, 33 out of 37 (89%) showed orcein-positive and rubeanic acid- positive intrahepatocytic copper granules. In the 4 negative cases conventional staining methods revealed characteristic bile duct lesions and granuloma formations; all 4 were so-called presymptomatic patients. Amongst the liver biopsy samples from CAH patients, 12 out of 22 (55%) contained orcein-positive granules, but none showed stainable copper. We conclude that (1) combined orcein and rubeanic acid positivity in liver samples helps to distinguish between PBC and CAH; (2) in presymptomatic PBC the negativity of both methods is adequately compensated by a high frequency of characteristic bile duct lesions and granuloma formation: (3) the absence of orceinophilic intrahepatocytic granules, in our hands, does not accurately predict the response to steroid therapy.