Case report: a common presentation of a rare disease-hepatosplenic T-cell lymphoma.
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2004-11
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63
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11
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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
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