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Sézary Syndrome in a 17‐Year‐Old Boy: Clinicopathologic Features and Genomic Profile |
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| Authors: | Silvia Alberti‐Violetti MD Pamela Vezzoli MD PhD Laura Corti PhD Daniele Fanoni MSc Valentina Merlo MSc Luigia Venegoni MLT Alberto Reseghetti MD Emilio Berti MD |
| Institution: | 1. Unità Operativa Complessa Dermatologia, Fondazione Istituto Di Ricovero e Cura a Carattere Scientifico Ca’ Granda—Ospedale Maggiore Policlinico, Milan, Italy;2. Unità Strutturale Complessa Dermatologia, Azienda Socio‐Sanitaria Territoriale Papa Giovanni XXIII, Bergamo, Italy;3. Dipartimento di Fisiopatologia Medico‐Chirurgica e dei Trapianti, Università degli Studi di Milano, Milan, Italy;4. Dipartimento di Scienze della Salute, Università degli Studi di Milano‐Bicocca, Milan, Italy |
| Abstract: | 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. |
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