An Atypical Case of Hepatosplenic T Cell Lymphoma

Hepatosplenic T cell lymphoma (HSTL) is a rare, aggressive subtype of extra-nodal lymphoma characterized by hepatosplenic presentation without lymphadenopathy that is typically seen in adolescents and young adults with a male predominance with bone marrow involvement. We present a case of a 55 year old woman with past history of pulmonary embolism, deep venous thrombosis and type II diabetes who complained of diffuse abdominal pain (worst in the LUQ) and lightheadedness. Ultimately, the patient was diagnosed with splenic hematoma and hemoperitoneum. This diagnosis necessitated splenectomy which revealed a markedly enlarged and ruptured spleen. Microscopic analysis with immunohistochemical stains showed a prominent abnormal T-cell population involving the splenic red pulp consistent with HSTL in this patient. Follow-up bone marrow biopsy was negative for involvement. We present this patient’s case due to her unique demographic and the atypical clinical features of her disease including lack of bone marrow presentation.