title: Hepato-splenic lymphoma: a rare entity mimicking acute hepatitis: a case report.
abstract: We reported a case of non-Hodgkin's lymphoma where liver involvement was the predominant clinical manifestation. A 27-year old man presented with markedly elevated serum aspartate aminotrasferase, alanine aminotransferase and lactate dehydrogenase, reduced prothrombin activity, thrombocytopenic purpura and hepato-splenomegaly without adenopathy. Viral, toxic, autoimmune and metabolic liver diseases were excluded. Bone marrow biopsy showed an intracapillary infiltration of T-lymphocytes with no evidence of lipid storage disease. Because of a progressive spleen enlargement, splenectomy was performed. Histological examination showed lymphomatous intrasinuses invasion of the spleen. Immunohistochemical investigation revealed the T phenotype of the neoplastic cells: CD45+, CD45RO+, CD3+, CD4-, CD8-, TIA1-. About 50 % of the lymphoid cells expressed CD56 antigen. The diagnosis of hepatosplenic T cell lymphoma was done. The patient was treated with chemotherapy, which induced a complete remission. Eighteen months later, he had a first relapse with increased aspartate aminotransferase, alanine aminotransferase, lactate dehydrogenase, thrombocytopenic purpura and blast in the peripheral blood. In spite of autologous bone marrow transplantation, he died twenty months after the diagnosis. Even in the absence of a mass lesion or lymphoadenopathy, hepatosplenic T-cell lymphoma should be considered in the differential diagnosis of a patient whose clinical course is atypical for acute hepatic dysfunction.
authors.affiliation: Department of Internal Medicine, University of Florence, Viale Pieraccini, 18, Zip Code 50139 Firenze, Italy.
authors.forename: Federico
authors.initials: F
authors.lastname: Perfetto
authors.forename: Roberto
authors.initials: R
authors.lastname: Tarquini
authors.forename: Francesco
authors.initials: F
authors.lastname: Mancuso
authors.forename: Simonetta
authors.initials: S
authors.lastname: Di Lollo
authors.forename: Silvia
authors.initials: S
authors.lastname: Tozzini
authors.forename: Giampiero
authors.initials: G
authors.lastname: Bellesi
authors.forename: Giacomo
authors.initials: G
authors.lastname: Laffi
mesh_terms: D000208:Acute Disease; D000328:Adult; D003937:Diagnosis, Differential; D006505:Hepatitis / Q000175:diagnosis*; D006801:Humans; D008113:Liver Neoplasms / Q000175:diagnosis*; D008228:Lymphoma, Non-Hodgkin / Q000175:diagnosis*; D008297:Male; D013160:Splenic Neoplasms / Q000175:diagnosis*