Royal academy of medicine in Ireland section of medicine

A 45 year old man with a 5 year history of primary biliary cirrhosis presented with fatigue, weight loss, bone pains, itch and progressive jaundice. Examination revealed jaundice, hyperpigmantadon, cachexin, extensive xanthelasm a and palmar xanthomata, spider naevi, palmar erythema, moderate hepatosplenomegaly, testicnlar atrophy and a proximal myopathy. Investigations revealed a uholestatie liver enzyme profile, hyperbilirubinaemia (73 umoU1), hypoalbuminaemia and profound hypercholesterolaemia (cholesterol 54.5 mmol/l). Antimitochondrial antibodies(M2) were positive at a titre of 1/ 10,240. A liver biopsy revealed grade III primary biliary cirrhosis, for which he was commenced on ursodeoxycbelie acid. Ultrasound of the abdomen revealed several descrete splenic nodules. CT abdomen noted an enlarged lymph node in the porte hepatis. A splenectomy was performed. Pathology revealed an intermediate grade follicular large cell non-Hodgkin's lympbema. Post-operatively, he received six courses of chemotherapy. The serum cholesterol fell sharply to 10 mmol/l after starting ursodeoxycholie acid. This dramatic fall in cholesterol represents an important beneficial effect of ursodeoxycholic acid therapy. It underlines the potential benefits of ursodeoxycholiu acid as a lipid lowering agent in cases of hypercholesterolemia due to impaired live~ cholesterol homeostasis. A review of the literature suggests that non-Hodgkin's lymphoma complicating primary biliary cirrhosis is very rare. It is suggested that lympbema may complicate autoimmune disease as a result of impaired immunoregulation or chronic antigen stimulation. In summary, this case highlights two important findings; firstly, the effect of ursodeoxycholic acid on hepatic cholesterol metabolism, and secondly, the association between primary biliary cirrhosis and non-Hodgkin's lympfioma.