Correlations of unique clinical, immunotypic, and histologic findings in cutaneous gamma/delta T-cell lymphoma

J Am Acad Dermatol. 1992 May;26(5 Pt 2):865-70. doi: 10.1016/0190-9622(92)70125-y.

Abstract

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.

Publication types

  • Case Reports

MeSH terms

  • Antigens, CD / analysis*
  • Antigens, CD / immunology
  • Antigens, Differentiation, T-Lymphocyte / analysis*
  • Antigens, Differentiation, T-Lymphocyte / immunology
  • Humans
  • Immunophenotyping / methods
  • Lymphoma, T-Cell, Cutaneous / diagnosis*
  • Lymphoma, T-Cell, Cutaneous / drug therapy
  • Lymphoma, T-Cell, Cutaneous / immunology
  • Lymphoma, T-Cell, Cutaneous / pathology
  • Male
  • Middle Aged
  • Receptors, Antigen, T-Cell, gamma-delta / analysis*
  • Receptors, Antigen, T-Cell, gamma-delta / immunology
  • Skin / pathology
  • Skin Neoplasms / diagnosis*
  • Skin Neoplasms / drug therapy
  • Skin Neoplasms / immunology
  • Skin Neoplasms / pathology
  • Staining and Labeling / methods

Substances

  • Antigens, CD
  • Antigens, Differentiation, T-Lymphocyte
  • Receptors, Antigen, T-Cell, gamma-delta