Atopic dermatitis-like non-erythrodermic leukemic variant of CD3(-/+dim) CD4(+) cutaneous T-cell lymphoma preceded by cutaneous papular xanthomatosis

• Type: Article in International Scientific Journal
• Date: 2004

Authors

Lima, M (Author) Other; Velho, G (Author) Other; Alves, R (Author) Other; Cunha, M (Author) Other; Teixeira, MD (Author) Other; Canelhas, A (Author) Other; Almeida, J (Author) Other; Sachse, F (Author) Other; Queiros, ML (Author) Other; Santos, AH (Author) Other; Fonseca, S (Author) Other; Goncalves, V (Author) Other; Massa, A (Author) Other; Orfao, A (Author) Other; Justica, B (Author) Other

Journal

Title: LEUKEMIA & LYMPHOMA

Vol. 45

Pages: 597-603

ISSN: 1042-8194

Publisher: INFORMA HEALTHCARE

Indexing

ISI Web of Knowledge - 6 Citations

Scopus - 7 Citations

Other information

Authenticus ID: P-000-BFF

DOI: 10.1080/10428190310001603939

Abstract (EN): 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.

Language: English

Type (Professor's evaluation): Scientific

No. of pages: 7