Primary cutaneous gamma/delta T-cell lymphoma presenting as disseminated pagetoid reticulosis.
The first case of primary gamma/delta cutaneous T-cell lymphoma (CTCL) with a fatal outcome is reported. The patient had the clinical and histopathologic features of disseminated pagetoid 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 gamma/delta variant of the T-cell receptor (CD3+, TCR-delta-1+) and were CD5+, CD7+, CD27+, CD29+, CD43+, CD44+, CD45+, CD45RA+, CD54+, CD69+, but beta F1-, Ti gamma a-, 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 gamma/delta 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 gamma/delta 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 gamma/delta T lymphocytes.
Berti
E
E
Istituto di Clinica Dermatologica Prima e Dermatologia Pediatrica, University of Milan, Italy.
Cerri
A
A
Cavicchini
S
S
Delia
D
D
Soligo
D
D
Alessi
E
E
Caputo
R
R
D000368:Aged; D015703:Antigens, CD / Q000032:analysis; D006801:Humans; D016399:Lymphoma, T-Cell / Q000276:immunology / Q000473:pathology*; D008297:Male; D010641:Phenotype; D011948:Receptors, Antigen, T-Cell / Q000032:analysis*; D016692:Receptors, Antigen, T-Cell, gamma-delta; D012867:Skin / Q000473:pathology*; D012878:Skin Neoplasms / Q000276:immunology / Q000473:pathology*
D015703:Antigens, CD; D011948:Receptors, Antigen, T-Cell; D016692:Receptors, Antigen, T-Cell, gamma-delta