Hepatosplenic .GAMMA..DELTA.T-cell Lymphoma: Report of a Case.

Abstract

A case of γδ T-cell lymphoma with severe hepatosplenomegaly was reported. A 17-year-old male was admitted to the Mie university hospital in Nov., 1991, because of jaundice and hepatosplenomegaly. Neither lymphadenopathy nor skin rush was detected. Laboratory data showed mild anemia, thrombocytopenia, and elevated serum lactate dehydrogenase (LDH) level (1338IU). Leucocyte count was 4790/μl with no abnormal finding. Bone marrow aspiration showed that nucleated cell count was 22.9×104/μl with 52.8% abnormal cells. The cells were large and had irregular nuclei and abundant cytoplasm without azulophilic granule. Their phenotype was CD3+ CD4- CD8- WT31- TCRδ1+ TdT-. DNA analysis of bone marrow mononuclear cells revealed rearrangements of T-cell receptor (TCR) γ and δ chain genes, and germline configulation in TCRβ chain gene and immunoglobulin heavy chain gene. He was diagnosed as γδ T-cell lymphoma (clinical stage IV B), and was first treated with combination therapy of CHOP and granulocyte-colony stimulating factor (G-CSF). The hepatosplenomegaly slightly regressed, but the abnormal cells in bone marrow did not decreased. The therapy was switched to one with carboplatin, ifosfamide, etoposide, and dexamethasone. The hepatosplenomegaly disappeared and the abnormal findings of bone marrow returned to normal. He is alive with partial response disease 7 months after the diagnosis.