title: Sézary Syndrome in a 17-Year-Old Boy: Clinicopathologic Features and Genomic Profile.
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.
authors.affiliation: Unità Operativa Complessa Dermatologia, Fondazione Istituto Di Ricovero e Cura a Carattere Scientifico Ca' Granda-Ospedale Maggiore Policlinico, Milan, Italy. silvia.viole@gmail.com.
authors.forename: Silvia
authors.initials: S
authors.lastname: Alberti-Violetti
authors.affiliation: Unità Strutturale Complessa Dermatologia, Azienda Socio-Sanitaria Territoriale Papa Giovanni XXIII, Bergamo, Italy.
authors.forename: Pamela
authors.initials: P
authors.lastname: Vezzoli
authors.affiliation: Unità Operativa Complessa Dermatologia, Fondazione Istituto Di Ricovero e Cura a Carattere Scientifico Ca' Granda-Ospedale Maggiore Policlinico, Milan, Italy.
authors.forename: Laura
authors.initials: L
authors.lastname: Corti
authors.affiliation: Unità Operativa Complessa Dermatologia, Fondazione Istituto Di Ricovero e Cura a Carattere Scientifico Ca' Granda-Ospedale Maggiore Policlinico, Milan, Italy.
authors.forename: Daniele
authors.initials: D
authors.lastname: Fanoni
authors.affiliation: Unità Operativa Complessa Dermatologia, Fondazione Istituto Di Ricovero e Cura a Carattere Scientifico Ca' Granda-Ospedale Maggiore Policlinico, Milan, Italy.
authors.forename: Valentina
authors.initials: V
authors.lastname: Merlo
authors.affiliation: Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Università degli Studi di Milano, Milan, Italy.
authors.forename: Luigia
authors.initials: L
authors.lastname: Venegoni
authors.affiliation: Unità Strutturale Complessa Dermatologia, Azienda Socio-Sanitaria Territoriale Papa Giovanni XXIII, Bergamo, Italy.
authors.forename: Alberto
authors.initials: A
authors.lastname: Reseghetti
authors.affiliation: Unità Operativa Complessa Dermatologia, Fondazione Istituto Di Ricovero e Cura a Carattere Scientifico Ca' Granda-Ospedale Maggiore Policlinico, Milan, Italy.|Dipartimento di Scienze della Salute, Università degli Studi di Milano-Bicocca, Milan, Italy.
authors.forename: Emilio
authors.initials: E
authors.lastname: Berti
mesh_terms: D000293:Adolescent; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001707:Biopsy, Needle; D003841:Deoxycytidine / Q000031:analogs & derivatives* / Q000627:therapeutic use; D023281:Genomics; D006801:Humans; D007150:Immunohistochemistry; D007558:Italy; D008297:Male; D011379:Prognosis; D012720:Severity of Illness Index; D012751:Sezary Syndrome / Q000188:drug therapy / Q000235:genetics* / Q000473:pathology*; D012878:Skin Neoplasms / Q000188:drug therapy / Q000235:genetics* / Q000473:pathology*; D016896:Treatment Outcome; D000093542:Gemcitabine
chemical_list: D000970:Antineoplastic Agents; D003841:Deoxycytidine; D000093542:Gemcitabine