A 36-year-old woman with a 2-week history of fever and markedly elevated lactate dehydrogenase levels. Nonenhanced CT and contrast-enhanced CT showed hepatosplenomegaly, diffusely decreased attenuation of the liver, and homogeneous enhancement in the hepatic and splenic parenchyma. F-FDG PET/CT revealed diffuse intense heterogeneous uptake by the liver (like superscan in bone scintigraphy). A liver needle biopsy confirmed the diagnosis of hepatosplenic alphabeta T-cell lymphoma. Subsequently, she received chemotherapy. The follow-up F-FDG PET/CT showed decreased F-FDG uptake in the liver and spleen.

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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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Mature T/NK-cell lymphomas (MTCLs) are a heterogeneous group of lymphoproliferative disorders, relatively rare in adults and children. Allogeneic hematopoietic stem cell transplantation (HSCT) can be considered in some cases as a consolidation and is the first choice for refractory forms and relapses. We retrospectively analyzed 19 pediatric patients with MTCL who received allogeneic hematopoietic stem cell transplantation from a haploidentical or unrelated donor on the αβ T cell depletion platform.

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