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.
|Titolo:||Primary cutaneous gamma/delta T-cell lymphoma presenting as disseminated pagetoid reticulosis|
|Parole Chiave:||Woringer-kolopp disease; monoclonal-antibodies; epidermal-cells; receptor; expression; lymphocytes; antigen; subpopulations; lieage; chains|
|Settore Scientifico Disciplinare:||Settore MED/35 - Malattie Cutanee e Veneree|
|Data di pubblicazione:||1991|
|Digital Object Identifier (DOI):||10.1111/1523-1747.ep12470953|
|Appare nelle tipologie:||01 - Articolo su periodico|