Hepatosplenic αβ T-cell lymphoma with myelodysplastic syndrome

We describe a patient with hepatosplenic up T-cell lymphoma who showed pancytopenia and myelodysplasia. A 35-yearold man was admitted with fever, pancytopenia, and hepatosplenomegaly but with no lymphadenopathy. We also found trilineage myelodysplasia in the bone marrow on his first admission. The patient had high fever and anemia but no evidence of infection and was tentatively treated with prednisolone. Ibis treatment resulted in a transient improvement of the cytopenia, and a reduction of spleen size. However, 10 months after the first manifestation, progression of the splenomegaly and fever became apparent, and a splenectomy was performed. The pathologic findings for the spleen showed diffuse and disseminated infiltration of medium- to large-sized T-lymphocytes in the splenic red pulp. These cells were immunohistochemically positive for CD3, CD5, CD7, CD8, CD16, CD56,T-cell receptor up (TCR alpha beta),T-cell intracellular antigen 1, and granzyme B but were negative for CD4, CD30, CD57, and TCR gamma delta These data suggested a diagnosis of hepatosplenic up T-cell lymphoma. A Southern blot analysis revealed gene rearrangement of the TCR beta-chain gene but not the immunoglobulin heavy chain gene in the spleen cells. An in situ hybridization analysis for the Epstein-Barr virus revealed negative results. The patient received 8 courses of combination chemotherapy and achieved a partial remission; however, the dysplastic features of the marrow cells persisted after the partial remission was obtained. Additional treatment with allogeneic bone marrow transplantation resulted in a transient complete remission; however, the patient relapsed 11 months later. Because he had experienced no lymphadenopathy and showed dysplastic features in the bone marrow, the diagnosis was highly dependent on the pathologic findings for the resected spleen.