Difficulty in diagnosis of a very rare tumour: hepatosplenic γδ T-Cell lymphoma that presented as an acute intestinal obstruction

Abstract

Introduction Hepatosplenic γδ T-cell lymphoma is a rare type of aggressive lymphoma predominantly affecting the liver and spleen. Case report We present a 19-year-old man admitted with fever, pancytopenia and acute intestinal obstruction. During emergency laparotomy he was found to have hepatosplenomegaly with lymphadenopathy along the mesentery. The spleen was enlarged and had compressed on the large bowel. The red pulp of the spleen was replaced by atypical lymphoid cells in a sinusoidal and perisinusoidal distribution. They were reactive to CD3, CD4, CD56, and TIA but negative to CD20, CD79a, CD5, CD57 and BF1. PCR analysis for clonal T-cell receptor gamma chain gene rearrangement studies was attempted from the paraffin blocks of the spleen. It was diagnosed as hepatosplenic γδ T-cell lymphoma. However, the patient defaulted and was readmitted 8 months later with pancytopenia, fever and jaundice. He developed leukaemic involvement of the bone marrow and succumbed to the illness 2 weeks later. Discussion The clinical presentation, morphology and its various immunohistochemical stains characteristic of hepatosplenic γδ T-cell lymphoma are discussed together with the problems and difficulty encountered in confirming the diagnosis. Hepatosplenic γδ T-cell lymphoma is aggressive and median survival is less than a year following diagnosis, despite treatment.