Hepatosplenic T-cell lymphoma of alpha beta lineage in a 16-year-old boy presenting with hemolytic anemia and thrombocytopenia

The authors report an unusual case of peripheral T-cell lymphoma in a 16-ye ar-old boy who presented initially with jaundice, splenomegaly, anemia, and thrombocytopenia. A lymphoma was found subsequently in the spleen, which w as infiltrated extensively in the red pulp by medium-sized, blastic-appeari ng lymphoma cells. Immunologic characterization of these cells revealed pos itivity for CD3, CD5, CD45RO, CD56, and T-cell intracellular antigen (TIA), and negativity for CD2, CD3, CD4, CD8, CD57, CD34, and terminal deoxynucle otidyl transferase (TdT). Conventional cytogenetic studies revealed the pre sence of isochromosome 7q. On follow up, this patient deteriorated rapidly, with evidence of liver and bone marrow involvement. Although the overall c linical and pathologic features of this disease were characteristic of hepa tosplenic gamma delta T-cell lymphoma, the T-cell receptor of this tumor sh owed an immunophenotype of alpha beta not gamma delta lineage. Using the So uthern blot technique, the authors demonstrated monoclonal gene rearrangeme nt of the T-cell receptor beta-chain. Thus, they confirmed the existence of hepatosplenic alpha beta T-cell lymphoma. In view of its overall similarit y to hepatosplenic gamma delta T-cell lymphoma, this unusual entity probabl y represents a slight biologic variation of the same disease.