Abstract

Hepatosplenic T cell lymphoma is a rare neoplasm accounting for less than 1 percent of non-Hodgkin lymphomas. It most commonly presents in young men with a median age at diagnosis of 35 years. Ten to 20 percent of patients have a history of chronic immune suppression related to treatment for solid organ transplantation, lymphoma, or inflammatory bowel disease. Most patients with HSTCL present with marked hepatosplenomegaly and thrombocytopenia without lymphadenopathy. Liver involvement is usually characterized by infiltration of dilated sinusoids by atypical lymphoid cells, with portal tracts and hepatocytes generally remaining uninvolved. Our patient presented with severe obstructive jaundice, impending hepatic failure and malignant ascites. He was refractory to steroid and radiation and biliary stenting had to be performed for severe hyperbilirubinemia.

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