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Hepatosplenic T-cell lymphoma is a rare histologic type of peripheral T-cell lymphomas, clinically characterized by predominant involvement of liver and spleen, with no or little adenopathy, and an often aggressive course. A 17 years old man presented with elevated serum aspartate aminotransferase, alanine aminotransferase and lactate dehydrogenase and hepatosplenomegaly without lymphadenopathy. Viral, toxic and autoimmune diseases were excluded. Hemochromatosis was suggested because of elevated serum ferritin, transferrin saturation and mildly decreased signal density in liver MRI. But biopsies of the liver and bone marrow revealed the infiltration of atypical T-lymphocyte with the immunophenotypic marker of CD3(+), CD4(-), CD8(-), CD56(-) and TIA-1(+). The iron deposits in hepatocytes were not seen. The study of chromosomal analysis revealed the normal findings. Thus, we could make a confirmative diagnosis as hepatosplenic T-cell lymphoma.(Korean J Med 70:S151-S155, 2006)
Key Word : Hepatosplenic lymphoma, T-cell, Hemochromatosis |
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