Treatment of Hepatosplenic T-Cell Lymphoma

Hepatosplenic T-cell lymphoma (HSTCL) is a rare subtype of peripheral T-cell lymphoma (PTCL) comprising approximately 1% of PTCL. HSTCL often involves young men. HSTCL appears to be localized to the hepatic and splenic sinusoids, with cell surface expression of the gamma delta T-cell receptor.[1-3] Characteristic chromosomal abnormalities such as isochromosome 7q and trisomy 8 are also suggestive of the diagnosis.[4] While in most cases the neoplastic cells express a gamma delta T-cell receptor (hepatosplenic gamma delta T-cell lymphoma), there are reports of alpha beta T-cell receptor expression (hepatosplenic alpha beta T-cell lymphoma).[5] This lymphoma has an extremely poor prognosis and an extremely aggressive clinical course.