Correlations of unique clinical, immunotypic, and histologic findings in cutaneous gamma/delta T-cell lymphoma.
Cutaneous T-cell lymphoma is a malignancy of T cells that express a clone-specific heterodimer T-cell receptor for antigen. The second recognized case of an epidermotropic malignancy of T-cells expressing gamma/delta T-cell receptor-expressing cells is reported. The immunophenotype of the malignant T-cells was CD3+, CD2+, CD7+, gamma/delta T-cell receptor+, CD4-, CD8-, and alpha/beta T-cell receptor-. The clinical features were remarkable for extreme epidermotropism with a scant dermal lymphomatous infiltrate. Profound keratinocyte necrosis occurred in areas of malignant skin infiltrates. Despite cutaneous lesions covering more than 50% of the skin surface of the patient, no adenopathy or splenomegaly was detected. The intense epidermotropism of this patient's gamma/delta T-cell receptor-expressing cells and the putative cytolytic properties of CD4- CD8- gamma/delta contributed to the destruction of epidermis. Remission was induced with a combination of electron beam and extracorporeal photochemotherapy.
Heald
P
P
Department of Dermatology, Yale University School of Medicine, New Haven Connecticut.
Buckley
P
P
Gilliam
A
A
Perez
M
M
Knobler
R
R
Kacinski
B
B
Edelson
R
R
D015703:Antigens, CD / Q000032:analysis* / Q000276:immunology; D000945:Antigens, Differentiation, T-Lymphocyte / Q000032:analysis* / Q000276:immunology; D006801:Humans; D016130:Immunophenotyping / Q000379:methods; D016410:Lymphoma, T-Cell, Cutaneous / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000032:analysis* / Q000276:immunology; D012867:Skin / Q000473:pathology; D012878:Skin Neoplasms / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D013194:Staining and Labeling / Q000379:methods
D015703:Antigens, CD; D000945:Antigens, Differentiation, T-Lymphocyte; D016692:Receptors, Antigen, T-Cell, gamma-delta