TY - JOUR
T1 - Primary cutaneous γ/δ T-cell lymphoma presenting as disseminated pagetoid reticulosis
AU - Berti, Emilio
AU - Cerri, Amilcare
AU - Cavicchini, Stefano
AU - Delia, Domenico
AU - Soligo, Davide
AU - Alessi, Elvio
AU - Caputo, Ruggero
PY - 1991/5
Y1 - 1991/5
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=0025868947&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0025868947&partnerID=8YFLogxK
M3 - Article
C2 - 1827136
AN - SCOPUS:0025868947
VL - 96
SP - 718
EP - 723
JO - Journal of Investigative Dermatology
JF - Journal of Investigative Dermatology
SN - 0022-202X
IS - 5
ER -