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

Area of Science:

• Hematology
• Oncology
• Immunology

Background:

• Hepatosplenic T-cell lymphoma (HSTCL) is a rare and aggressive hematologic malignancy.
• Most HSTCL cases express gamma-delta T-cell receptors (TCRs), while a rarer subset expresses alpha-beta TCRs.
• Alpha-beta TCR HSTCL cases may exhibit unique clinical and morphological traits.

Observation:

• This report describes an alpha-beta TCR HSTCL case with a hemolytic presentation and aggressive clinical course.
• Morphological analysis revealed diffuse growth, blastoid morphology, and CD8+ positivity against a CD4+ T-cell background.
• Tumor cells showed periportal localization in the liver and emperipolesis in the bone marrow.

Findings:

• The observed emperipolesis may contribute to the pancytopenia characteristic of HSTCL.
• The alpha-beta HSTCL case presented with hemolytic anemia, unrelated to immunosuppression.
• Distinct morphological features included blastoid cells and specific T-cell receptor expression.

Implications:

• The unusual presentation highlights diagnostic challenges for this rare HSTCL subtype.
• Further case studies are necessary to determine if alpha-beta and gamma-delta HSTCL are distinct entities prognostically or morphologically.
• Understanding these distinctions is crucial for accurate diagnosis and treatment strategies in HSTCL.