Atopic dermatitis-like non-erythrodermic leukemic variant of CD3(-/+dim) CD4(+) cutaneous T-cell lymphoma preceded by cutaneous papular xanthomatosis.
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.
Lima
Margarida
M
Service of Clinical Hematology, Hospital Geral de Santo António, Porto, Portugal. mmc.lima@clix.pt
Velho
Glória
G
Alves
Rosário
R
Cunha
Manuel
M
Teixeira
Maria dos Anjos
Mdos A
Canelhas
Aurea
A
Almeida
Julia
J
Sachse
Fernanda
F
Queirós
Maria Luís
ML
Santos
Ana Helena
AH
Fonseca
Sonia
S
Gonçalves
Vicente
V
Massa
António
A
Orfão
Alberto
A
Justiça
Benvindo
B
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*
D017252:CD3 Complex; D018793:Interleukin-13