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Thiopurine drugs, azathioprine (Imuran) and 6-mercaptopurine (6-MP), are immunomodulators that have been shown to be effective at inducing and maintaining remission in inflammatory bowel disease. Although usually well-tolerated, the occurrence of side effects, typically myelotoxicity and hepatotoxicity, is a major drawback. The side effects can be classified as dose-dependent and independent. Both cholestatic hepatitis and endothelial injury, leading to vascular congestion and nodular regenerative hyperplasia, have been described during therapy with thiopurines, which can end up with portal hypertension. These injuries are potentially mediated by different metabolites. In this article we present a case of hyperammonaemic encephalopathy during therapy with 6-MP, possibly the first recorded in the literature, which probably resulted from the combination of thiopurine-induced liver injury with portal hypertension and the presence of spontaneous portosystemic venous shunts.

A 48 years old patient was admitted to the Internal Medicine ward due to progressive weakness and abnormal liver function tests. During three months of hospitalization she developed opportunistic infections with Cryptococcus and Pneumocystic jiroveci pneumonia. The CD4+ T-cell lymphocyte count was very low with no evidence of infection with human immunodeficiency virus. Liver disease deteriorated with the appearance of profound jaundice and severe hepatitis. The patient's laboratory and clinical presentation were compatible with the diagnosis of idiopathic CD4 + T-cell lymphocytopenia--ICL. The authors reviewed the literature on ICL and discuss the rare hepatic presentation of this uncommon syndrome.