Intraclonal diversity in a Sezary syndrome with a differential response to 2-deoxycoformycin of the two lymphoma cell populations.
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.
Granjo
Elisa
E
Department of Clinical Haematology, Hospital Geral de São João, Porto, Portugal. npp46740@mail.telpac.pt
Lima
Margarida
M
Lopes
José Manuel
JM
Cunha
Nória
N
Teixeira
Maria Dos Anjos
Mdos A
Santos
Filipe
F
Candeias
Jorge
J
Resende
Carlos
C
Santos
Ana Helena
AH
Balanzategui
Ana
A
Orfão
Alberto
A
Matutes
Estella
E
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*
D000903:Antibiotics, Antineoplastic; D015649:Pentostatin