Hepatosplenic γδ T Cell Lymphoma Involving the Brain

Abstract

Background: Brain involvement of hepatosplenic T cell lymphoma (HSTL) has not been reported so far.

Case description: We observed an extremely rare case of HSTL, which is a rare and aggressive variant of peripheral T cell lymphoma, generally showing predominant infiltration to the liver, spleen, and bone marrow and involving the brain. A 41-year-old Japanese woman presented with dysarthria and numbness of the right hand. Radiologic examination revealed a single 3-cm mass in the left frontal cortex, which was totally removed. Pathologic examination of the specimen demonstrated T cell lymphoma with a γδ cytotoxic T cell phenotype. Multiplex polymerase chain reaction analyses confirmed monoclonality of T cell receptor γ. Systemic examination revealed infiltration of atypical T lymphoid cells of the same phenotype in bone marrow and the presence of hepatosplenomegaly. We diagnosed HSTL involving the brain. The patient was treated with several courses of intensive chemotherapy, but it failed to achieve remission. She died of sepsis 4 months after the surgery.

Conclusions: HSTL can involve the brain. A diagnosis of HSTL involving the brain needs careful systemic evaluation. Timely and precise diagnosis that considers the systemic condition is important for appropriate treatment and better outcome.

Keywords: Brain involvement; Liver; Lymphoma; Spleen; T cell.