Atopic Dermatitis-Like Non-Erythrodermic Leukemic Variant of CD3 -/+dim CD4 + Cutaneous T-Cell Lymphoma Preceded by Cutaneous Papular Xanthomatosis.

Autor: Lima, Margarida, Velho, Glória, Alves, Rosário, Cunha, Manuel, Teixeira, Maria Dos Anjos, Canelhas, Áurea, Almeida, Julia, Sachse, Fernanda, Queirós, Maria Luís, Santos, Ana Helena, Fonseca, Sónia, Gonçalves, Vicente, Massa, António, Orfão, Alberto, Justiça, Benvindo
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Zdroj: Leukemia & Lymphoma; Mar2004, Vol. 45 Issue 3, p597-603, 7p
Abstrakt: We report a patient with cutaneous papular xanthomatosis who 4 years later developed a CD3 -/+dim /CD4 + T-cell lymphoma. Pruritic xerotic non-erythrodermic skin, eosinophilia and hyper-IgE were present and erroneously classified as atopic dermatitis. Flow cytometry and DNA ploidy analysis of both blood and skin lymphocytes, skin histology and blood T-cell receptor gene rearrangement studies confirmed diagnosis of T-cell lymphoma. Monoclonal CD3 -/+dim /CD4 + T-cells were especially prone to the synthesis of IL-13, a cytokine that is involved in IgE-secretion, and comprised both a medium (diploid) and large (hyperploid) sized T-cell populations with a similar immunophenotype. The majority of the normal residual T-cells were large granular lymphocytes, expressed activation-related and natural-killer -associated markers and secreted high levels of interferon gamma, suggesting that they might correspond to active cytotoxic cells directed against the neoplastic T-lymphocytes. [ABSTRACT FROM AUTHOR]
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