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

J Hematol

April 2024

Department of Pathology, HCA East Florida, Fort Lauderdale, FL, USA.

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.

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The patient was a 21-year-old man who had been diagnosed with Crohn's disease and received infliximab and azathioprine six years earlier. He was admitted with fever and fatigue. Peripheral blood examination showed LDH 2,473 U/l and thrombocytopenia, and contrast-enhanced computed tomography (CT) showed hepatosplenomegaly.

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