title: Intraclonal diversity in a Sezary syndrome with a differential response to 2-deoxycoformycin of the two lymphoma cell populations.
abstract: We report a case of Sezary syndrome with two abnormal CD4+ T-cell populations detected in the peripheral blood by flow cytometry immunophenotyping and DNA cell content, suggesting a biclonal T-cell lymphoproliferative disorder. Despite these findings, molecular analysis of the T-cell receptor genes was consistent with a monoclonal T-cell proliferation, supporting the existence of intraclonal diversity rather than a true biclonal disease. The patient achieved a transient response with 2-deoxycoformycin, with a selective decrease of the larger/hyperploid T-cell population; later on, an increased representation of this T-cell population was observed concomitantly with clinical relapse.
authors.affiliation: Department of Clinical Haematology, Hospital Geral de São João, Porto, Portugal. npp46740@mail.telpac.pt
authors.forename: Elisa
authors.initials: E
authors.lastname: Granjo
authors.forename: Margarida
authors.initials: M
authors.lastname: Lima
authors.forename: José Manuel
authors.initials: JM
authors.lastname: Lopes
authors.forename: Nória
authors.initials: N
authors.lastname: Cunha
authors.forename: Maria Dos Anjos
authors.initials: Mdos A
authors.lastname: Teixeira
authors.forename: Filipe
authors.initials: F
authors.lastname: Santos
authors.forename: Jorge
authors.initials: J
authors.lastname: Candeias
authors.forename: Carlos
authors.initials: C
authors.lastname: Resende
authors.forename: Ana Helena
authors.initials: AH
authors.lastname: Santos
authors.forename: Ana
authors.initials: A
authors.lastname: Balanzategui
authors.forename: Alberto
authors.initials: A
authors.lastname: Orfão
authors.forename: Estella
authors.initials: E
authors.lastname: Matutes
mesh_terms: D000368:Aged; D000903:Antibiotics, Antineoplastic / Q000627:therapeutic use*; D015496:CD4-Positive T-Lymphocytes / Q000276:immunology*; D002455:Cell Division; D005434:Flow Cytometry; D006801:Humans; D016130:Immunophenotyping / Q000379:methods; D008297:Male; D015649:Pentostatin / Q000627:therapeutic use*; D010641:Phenotype; D012751:Sezary Syndrome / Q000188:drug therapy* / Q000276:immunology; D012878:Skin Neoplasms / Q000188:drug therapy* / Q000276:immunology; D016176:T-Lymphocyte Subsets / Q000276:immunology*
chemical_list: D000903:Antibiotics, Antineoplastic; D015649:Pentostatin