Primary cutaneous γ/δ T-cell lymphoma presenting as disseminated pagetoid reticulosis

Emilio Berti, Amilcare Cerri, Stefano Cavicchini, Domenico Delia, Davide Soligo, Elvio Alessi, Ruggero Caputo

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Abstract

The first case of primary γ/δ cutaneous T-cell lymphoma (CTCL) with a fatal outcome is reported. The patient had the clinical and histopathologic features of disseminated page told reticulosis, a rare form of CTCL characterized by a strong epidermotropic lymphoid infiltrate. Extensive immuno-cytochemical studies showed that the neoplastic cells were almost exclusively localized in the epidermis, expressed the γ/δ variant of the T-cell receptor (CD3+, TCR-δ-1+) and were CD5+, CD7+, CD27+, CD29+, CD43+, CD44+, CD45+, CD45RA+, CD54+, CD69+, but βF1-, Tiγa-, BB3-, A13-, CD2-, CD4-, CD8-, CD11a -, CD49d-, CD25-, CD30-, and HLA-DR-. A comparison of our results with those of the literature, which have not included γ/δ T-cell receptor analysis, suggests that some reported cases of pagetoid reticulosis may have phenotypes similar to our case. Electron microscopy studies demonstrated that the γ/δ T lymphocytes were villous, containing dense and multivesicular bodies, and formed close contacts with the surrounding keratinocytes, suggesting that these cells should have a role in the skin-associated lymphoid tissue. The proliferating cells in our case might represent the neoplastic counterpart of the recently reported CD2- subset of normal human peripheral blood γ/δ T lymphocytes.

Original languageEnglish
Pages (from-to)718-723
Number of pages6
JournalJournal of Investigative Dermatology
Volume96
Issue number5
Publication statusPublished - May 1991

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ASJC Scopus subject areas

  • Dermatology

Cite this

Berti, E., Cerri, A., Cavicchini, S., Delia, D., Soligo, D., Alessi, E., & Caputo, R. (1991). Primary cutaneous γ/δ T-cell lymphoma presenting as disseminated pagetoid reticulosis. Journal of Investigative Dermatology, 96(5), 718-723.