Hepatosplenic t-cell lymphoma masquerading as chronic liver disease: a rare clinical presentation

Hepatosplenic T-cell lymphoma (HSTCL) was first described as a distinct clinicopathologic entity in 19901 HSTCL is a rare type of non-Hodgkin lymphoma that was originally recognized by its characteristic clinical presentation, distinct histologic pattern, and expression of the ɤδ T-cell receptor (TCR)1.Recent scientific advances have allowed for better understanding of the histologic, immunophenotyping, and cytogenetic characteristics of HSTCL, including identification of HSTCL with αβ TCR expression. γδ HSTCL and αβ HSTCL are now considered immunophenotypic variants of the same disease. Despite these advances, HSTCL remains a very aggressive subset of T-cell lymphoma and confers a poor prognosis, with a reported median survival of 6–11 months2,3 .There has been no consensus to date regarding therapeutic modalities in these patients, and effective treatment of HSTCL is lacking. A review of the literature reveals the use of various treatment regimens in patients with HSTCL. The majority of these treatment modalities appear to be ineffective in most patients, although there are some case reports and case series that describe complete remissions with certain chemotherapy regimens with or without stem cell transplantation.2,4