History and clinical findings: 16 years ago a now 53-year-old woman was found to have primary biliary cirrhosis. 5 years later, after bleeding from oesophageal varices, she had a portacaval shunt. For several years she had been taking ursodeoxycholic acid (750 mg daily). Because of steadily increasing jaundice over the past few years she presented for possible liver transplantation.
Investigations: There was a discrepancy between the markedly raised serum bilirubin concentration (7.8 mg/dl) and the only slightly raised or normal activities of alkaline phosphatase (247 U/l) and gamma-GT (21 U/l). Further tests confirmed that the patients had not only PBC but also Coombs-negative haemolytic anaemia (haemoglobin 10.7 g/dl, reticulocyte count 122/1000, indirect bilirubin 6.4 mg/dl, haptoglobin not demonstrated, lactate dehydrogenase 316 U/l). She had splenomegaly despite the portacaval shunt. Blood smear revealed spherocytes, but hereditary spherocytosis was not confirmed.
Treatment and course: A six-week interruption of taking ursodeoxycholic acid led, as expected, to a rise in the activities of serum alkaline phosphatase and gamma-GT, while haemolysis parameters were not affected.
Conclusion: Serum bilirubin concentration is a decisive prognostic factor in the course of primary biliary cirrhosis and is therefore of particular relevance for the indication of liver transplantation. The reported case demonstrates the importance of considering other causes of hyperbilirubinaemia.