Sézary Syndrome in a 17‐Year‐Old Boy: Clinicopathologic Features and Genomic Profile

We describe the case of a 17‐year‐old Hispanic boy who had had erythroderma and diffuse lymphadenopathy for approximately 6 months. A diagnosis of Sézary syndrome was made on the basis of the histologic features of the skin; the presence of the same T‐cell clone on the skin, blood, and bone marrow;...

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Veröffentlicht in:Pediatric dermatology 2016-09, Vol.33 (5), p.e318-e321
Hauptverfasser: Alberti‐Violetti, Silvia, Vezzoli, Pamela, Corti, Laura, Fanoni, Daniele, Merlo, Valentina, Venegoni, Luigia, Reseghetti, Alberto, Berti, Emilio
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Sprache:eng
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Zusammenfassung:We describe the case of a 17‐year‐old Hispanic boy who had had erythroderma and diffuse lymphadenopathy for approximately 6 months. A diagnosis of Sézary syndrome was made on the basis of the histologic features of the skin; the presence of the same T‐cell clone on the skin, blood, and bone marrow; and the high CD4+ lymphocyte count with an aberrant phenotype in peripheral blood; bone marrow involvement was also present. The patient was treated with systemic gemcitabine and achieved partial remission.
ISSN:0736-8046
1525-1470
DOI:10.1111/pde.12930