Primary intestinal T-cell lymphoma and sclerosing cholangitis: a cytokine-mediated association?

A 63-year-old woman with a 1-year history of abdominal pain and intrahepati c cholestasis developed anorexia, weight loss, lassitude and diarrhoea, Stu dies led to a diagnosis of primary intestinal T-cell lymphoma involving esp ecially the proximal small intestine and infiltrating the mesenteric lymph nodes, bone marrow and skin. An associated severe hypoalbuminaemia (1.3 g d L(-1)) was most probably the result of protein-losing enteropathy. Liver bi opsy demonstrated concentric fibrosis of the bile ducts ('onion skin' lesio ns, with an inflammatory cell infiltrate and lymphoid aggregates) and was c onsidered almost pathognomonic of primary sclerosing cholangitis. Sudden de ath due to pulmonary embolism occurred and a limited autopsy confirmed the diagnosis. Other associated diseases such as coeliac disease or inflammator y bowel disease were not found. This first report of the simultaneous occur rence of two rare diseases - primary sclerosing cholangitis and intestinal T-cell lymphoma - may indicate an intriguing association, possibly mediated by the effect of cytokines released by the infiltrating T-cells into the p ortal circulation.