Abstract

Intravascular lymphoma is an exceedingly rare non-nodal variant of non-Hodgkin ?lymphoma, in which large pleomorphic neoplastic lymphocytes are found predominantly ?within vessels. Neoplastic lymphocytes in intravascular lymphoma are overwhelmingly of ?a B-cell phenotype and the disease is usually aggressive and widespread. Intravascular T-?cell lymphomas include those with an NK/T-cell phenotype, often with expression of ?EBV, and ALK- anaplastic large cell lymphoma. In this case report, we present the ?clinical course and autopsy findings of an unusual intravascular, CD4+ cytotoxic T-cell ?lymphoma without expression of EBV or CD30. An 81-year old man presented with a 3-?month history of indurated nodules on the thighs associated with weight loss, fevers, ?pleural effusion and osteolytic lesions. He subsequently developed angulated, retiform, ?palpable dusky plaques on the chest and shoulders with altered mental status and diffuse ?progressive weakness. Skin biopsy revealed an intravascular, cytotoxic T-cell lymphoma ?with expression of CD3, CD4, CD2, TIA-1, granzyme, and weak expression of CD56, ?CD5 and CD7. Intravascular T-cells expressed weak CD20, but did not express PAX5. ?EBV in situ hybridization (EBER), CD30 and ALK were negative. On autopsy, the ?lymphoma was widely disseminated, involving small- and medium-caliber vessels in the ?skin, bone, lungs, heart, spleen, lymph nodes, pituitary gland, adrenal glands, pancreas, ?thyroid and esophagus. This case adds to the small number of intravascular T-cell ?lymphomas reported in the literature and provides detailed clinical and pathologic ?information.?

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