Hepatosplenic Alpha-Beta T-Cell Lymphoma: A Challenging Diagnostic Entity

Hepatosplenic T-cell lymphoma (HSTCL) is rare and clinically very aggressive T-cell lymphoma. The majority of cases harbor γδ T-cell receptors (TCRs); however, in some even rarer cases, tumor cells harbor αβ TCR. Recent studies suggest that αβ cases may have distinct morphological characteristics and demonstrate an even more aggressive course. In this case report, we demonstrated that in line with previous findings, αβ case of HSTCL had hemolytic presentation, demonstrated a very aggressive clinical course, and was unrelated to immunosuppression. Morphologically, tumor cells demonstrated diffuse growth pattern, blastoid morphology, and were CD8+ positive on the background of CD4+ small to medium reactive T cells. Additionally, the liver tumor cells demonstrated periportal localization, and in bone marrow, evidence of emperipolesis was noted. The latter finding may significantly contribute to pancytopenia characteristic, all types of HSTCL. Those unusual morphologic and clinical characteristics make diagnosis of this rare subtype of rare disease very challenging. More case analysis is required to establish whether αβ/γδ HSTCL are prognostically or morphologically significantly distinct entities.