Cirrhotic process, liver cell carcinoma and extrahepatic malignant tumors in idiopathic haemochromatosis. Study of 71 patients treated with venesection therapy.

Abstract

A detailed morphological study concerning the liver of 71 patients suffering from idiopathic haemochromatosis was carried out. These patients were subjected to long-term venesection therapy, which lasted from 1 to 12 years. The mean duration of therapy was 7 years for each patient. The material consisted of needle biopsies of the liver. Three to seven biopsies were performed in each patient; the initial biopsy, the biopsy following venesection therapy, and the follow-up biopsies. In 30 cases the follow-up was performed in autopsy material. It was found that the final histological appearance of the liver remained unchanged in 44 (62%) cases, became worse in 23 (32%) cases, and improved in 4 (5%) cases. However, the change for the worse is generally retarded and consequently the mean survival of the patients is increased. Two out of 4 cases that showed improvement (i.e. changing from cirrhosis to fibrosis) are strongly supported by the fact that the follow-up study was performed in autopsy material after 2-5 needle liver biopsies. A high incidence of hepatocellular carcinoma (18%), as well as of a malignant neoplasia in another organ (8.4%), was noted in spite of the long-term venesection therapy. It follows therefore that the longer survival of patients with idiopathic haemochromatosis increases the possibility of hepatocellular carcinoma or of a malignancy in another organ.