|
|||||
|
|
Sézary Syndrome in a 17‐Year‐Old Boy: Clinicopathologic Features and Genomic Profile |
|
| Authors: | Silvia Alberti‐Violetti M.D. Pamela Vezzoli M.D. Ph.D. Laura Corti Ph.D. Daniele Fanoni M.Sc. Valentina Merlo M.Sc. Luigia Venegoni M.L.T. Alberto Reseghetti M.D. Emilio Berti M.D. |
| Affiliation: | 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. |
| Keywords: | |