Case report: a common presentation of a rare disease-hepatosplenic T-cell lymphoma.

Abstract

Hepatosplenic T-cell lymphoma is a rare neoplasm characterized by systemic B-symptoms, hepatosplenomegaly, no lymphadenopathy, and lymphomatous infiltrates in the splenic red pulp, hepatic sinusoids, and bone marrow sinuses. The team presents the case of a healthy 30 year old man, active duty Marine, who presented with classic symptoms, yet obtaining a diagnosis took over three months from the onset of symptoms. This clinical entity initially described in 1990, is elusive, with vague and misleading symptoms. Despite aggressive conventional therapy with anthracycline-based regimens and stem cell transplant, prognosis is poor and median survival is less than one year