[Wilson's disease. A histological review of 7 patients and the value of histological copper positivity in relation to other hepatopathies].

BACKGROUND

Wilson's disease is an infrequent entity. Biopsy is essential for establishing its presence and degree of hepatopathy but it lacks diagnostic specificity. It is necessary to know the significance of positivity or negativity of histochemically demonstrated copper when faced with the diagnosis of Wilson's disease and cholestatic diseases.

METHODS

Nine hepatic sample from 7 patients with clinical-biochemical Wilson's disease were reviewed. The rodanine histochemical technique was used for demonstration of copper in the samples aforementioned and in another 3 kinds of liver diseases: 16 biopsies of primary biliary cirrhosis, 86 biopsies of cholestatic hepatopathies (serum bilirubin greater than 68 mumol/l) and control biopsies with serum bilirubin less than 42.5 mumol/l.

RESULTS

Among the 7 patients with Wilson's disease, 6 were diagnosed with cirrhosis and one chronic active hepatitis. The incidence of erosive necrosis (86%), Mallory hyaline (86%), macrovacuolar steatosis (71%), glucogenated nuclei (71%) and giant mitochondria (29%) was evaluated. Histologic copper deposit with Shikata orcein was objectified in 3 cases (43%) and with rodanine in 5 (83%). Positivity with rodanine was 69% among the series of primary biliary cirrhosis, 20% among the cholestatic hepatopathies and 0 in the control series.

CONCLUSIONS

Histology of Wilson's disease remains without definitive diagnostic criteria. The disease is diagnosed in a late phase in Spain. The sensitivity of the histochemical demonstration of copper with rodanine is high (6/7) but its specificity is low, being positive in 68% of primary biliary cirrhosis and 19% of other cholestatic hepatopathies.