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.
Alberti Violetti, S., Vezzoli, P., Corti, L., Fanoni, D., Merlo, V., Venegoni, L., et al. (2016). Sézary Syndrome in a 17-Year-Old Boy: Clinicopathologic Features and Genomic Profile. PEDIATRIC DERMATOLOGY, 33(5), e318-e321 [10.1111/pde.12930].
Sézary Syndrome in a 17-Year-Old Boy: Clinicopathologic Features and Genomic Profile
BERTI, EMILIOUltimo
2016
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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