Histology of the liver in Wilson's disease: a study of 34 cases

Biopsy or autopsy samples of liver from 34 patients with clinical evidence of Wilson's disease were studied histologically. Seven had early changes ranging from slight pleomorphism of hepatocytes to fatty metamorphosis, vacuolated nuclei, and focal necrosis. Seven other specimens from cases classified as chronic active hepatitis were characterized by periportal degeneration and necrosis, in addition to many of the changes of the first group. Periportal copper accumulation was found in three of these specimens and was considered helpful in the distinction from other forms of chronic active hepatitis. Specimens from four of the precirrhotic cases showed periportal atypical lipofuscin granules. The 20 cirrhotic specimens were macronodular or mixed micronodular-macronodular and showed a wide variety of patterns and cytologic changes. Eight contained Mallory bodies. Copper was demonstrated in 13 of 15 specimens with adequate copper stains. Some of the findings described here may suggest or confirm a diagnosis of Wilson's disease, but none is considered specific. A proper approach to the diagnosis should correlate the clinical, laboratory, and morphologic findings.