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Images of Memorable Cases: Case 110

Module by: Herbert L. Fred, MD, Hendrik A. van Dijk

http://rup.rice.edu/memcases-button.jpg

Case_110-pres1-1.jpg Case_110-pres1-3.jpg

This 61-year-old man presented in 1972 with unrelenting pruritus of six months’ duration. On the right is his peripheral blood film stained with Periodic Acid-Schiff (PAS).

110. Sézary syndrome (SS)

A rare form of cutaneous lymphoma characterized by intractable pruritus, generalized erythroderma, diffuse lymphadenopathy, and neoplastic T cells (Sézary cells) in the skin, lymph nodes, and peripheral blood. The Sézary cells in the blood are mononuclear, usually numerous, and have round or oval nuclei with densely clumped chromatin. They often stain intensely with PAS (image below), but more sophisticated techniques are now available to identify them.

SS predominantly affects men over the age of 60. When the erythroderma is generalized, the term l’homme rouge (the red man) has been applied. Treatment currently includes photophoresis, with or without interferon, chemotherapy, and total skin electron beam therapy. Prognosis is poor with a median survival of two to four years.

The patient shown received prednisone and systemic chemotherapy. His skin manifestations initially improved, but he subsequently manifested widespread lymphadenopathy with hepatosplenomegaly, and died four years after onset of symptoms.

Case_110-pres1-2.jpg

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