title: Atopic dermatitis-like non-erythrodermic leukemic variant of CD3(-/+dim) CD4(+) cutaneous T-cell lymphoma preceded by cutaneous papular xanthomatosis.
abstract: 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.
authors.affiliation: Service of Clinical Hematology, Hospital Geral de Santo António, Porto, Portugal. mmc.lima@clix.pt
authors.forename: Margarida
authors.initials: M
authors.lastname: Lima
authors.forename: Glória
authors.initials: G
authors.lastname: Velho
authors.forename: Rosário
authors.initials: R
authors.lastname: Alves
authors.forename: Manuel
authors.initials: M
authors.lastname: Cunha
authors.forename: Maria dos Anjos
authors.initials: Mdos A
authors.lastname: Teixeira
authors.forename: Aurea
authors.initials: A
authors.lastname: Canelhas
authors.forename: Julia
authors.initials: J
authors.lastname: Almeida
authors.forename: Fernanda
authors.initials: F
authors.lastname: Sachse
authors.forename: Maria Luís
authors.initials: ML
authors.lastname: Queirós
authors.forename: Ana Helena
authors.initials: AH
authors.lastname: Santos
authors.forename: Sonia
authors.initials: S
authors.lastname: Fonseca
authors.forename: Vicente
authors.initials: V
authors.lastname: Gonçalves
authors.forename: António
authors.initials: A
authors.lastname: Massa
authors.forename: Alberto
authors.initials: A
authors.lastname: Orfão
authors.forename: Benvindo
authors.initials: B
authors.lastname: Justiça
mesh_terms: D000328:Adult; D017252:CD3 Complex / Q000032:analysis; D015496:CD4-Positive T-Lymphocytes / Q000473:pathology; D002471:Cell Transformation, Neoplastic; D003872:Dermatitis / Q000175:diagnosis*; D003873:Dermatitis, Exfoliative; D003937:Diagnosis, Differential; D006801:Humans; D018793:Interleukin-13 / Q000096:biosynthesis; D017254:Leukemic Infiltration; D016410:Lymphoma, T-Cell, Cutaneous / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology*; D008297:Male; D016176:T-Lymphocyte Subsets / Q000276:immunology; D014973:Xanthomatosis / Q000150:complications*
chemical_list: D017252:CD3 Complex; D018793:Interleukin-13