An unusual presentation of an uncommon lymphoma, hepatosplenic T-cell lymphoma

Abstract

Hepatosplenic T-cell lymphoma (HSTCL) is a rare T-cell lymphoma, primarily characterized by extranodal distribution of the malignant cells with intrasinusoidal infiltration of the liver, spleen, and bone marrow, which is associated with a poor outcome. We describe a unique case of a 47-year-old woman with a clinical presentation of headaches, fevers, elevated liver function tests, and hepatosplenomegaly. A liver biopsy revealed a striking hepatic intrasinusoidal infiltrate of lymphocytes. These were confirmed to be T cells by immunohistochemical stains (CD8⁺, CD4⁻, CD3⁺, CD20⁻). Molecular studies demonstrated a T-cell gamma receptor gene rearrangement. Clinically, the patient appeared to improve without therapeutic intervention; however, because of the pathologic diagnosis, the patient was initiated on chemotherapy and ultimately underwent a bone marrow transplant.

Keywords:

• Gamma-delta hepatosplenic T-cell lymphoma
• hepatosplenic T cell lymphoma
• HSTCL