Hepatosplenic T-cell Lymphoma : Clinical Features
Hepatosplenic T-cell lymphoma (HSTCL) is a disease of young adults, with a median age at diagnosis of 35 years. It presents with hepatosplenomegaly but no lymphadenopathy. Most patients have fever, fatigue, malaise, weakness, weight loss, and abdominal pain (from marked splenomegaly).
Bone marrow is usually involved causing cytopenias. Thrombocytopenia is common and may be misinterpreted as idiopathic thrombocytopenic purpura. About 50% of patients also have leukopenia and anemia. In spite of bone marrow involvement, there is no significant leukemic phase. There is no lymphocytosis; however, peripheral blood smears may show a small number of atypical lymphoid cells.
Chest films and CT scans show no mediastinal or retroperitoneal lymphadenopathy. The diagnosis is made on bone marrow biopsy which is preferred over splenectomy and liver biopsy. The expression of gamma-delta T-cell receptor can be confirmed by flow cytometry on a bone marrow sample.
This low magnification image of HSTCL in spleen shows expansion of red pulp due to diffuse infiltration of the cords and sinuses by the neoplastic cells with preservation of architecture. White pulp areas are reduced.