Two atypical cases of cutaneous gamma/delta T-cell lymphomas

Dermatology. 2011;222(4):297-303. doi: 10.1159/000327932. Epub 2011 May 21.

Abstract

Cutaneous γ/δ T-cell lymphoma (CGD-TCL) is a recent entity described in the newly revised World Health Organization-European Organization for Research and Treatment of Cancer classification of cutaneous lymphomas, and is characterized by the γ/δ T-cell receptor expression on atypical lymphocytes. Only a few cases of primary CGD-TCL have been reported, with an extremely aggressive course (median survival time of 15 months). We describe 2 atypical cases of CGD-TCL. The first case was initially misdiagnosed as an inflammatory panniculitis due to the granulomatous infiltrate on the skin biopsy specimen. Diagnosis was confirmed using δ PCR that revealed γ/δ T-cell clonal expansion. The evolution was marked by predominant γ/δ T-cell infiltrate with diffuse body fat involvement as seen on positron emission tomography-computed tomography. The second case is the first described Epstein-Barr virus (EBV)-associated CGD-TCL with a rapidly fatal evolution. CGD-TCL is also a heterogeneous entity and δ PCR and EBV-encoded RNA probe to detect an EBV latent infection may help diagnose and characterize these cutaneous lymphomas.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Aged
  • Antineoplastic Agents / therapeutic use
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Cyclophosphamide / therapeutic use
  • Doxorubicin / therapeutic use
  • Epstein-Barr Virus Infections / diagnosis
  • Fatal Outcome
  • Female
  • Humans
  • Lymphoma, T-Cell, Cutaneous / diagnosis*
  • Lymphoma, T-Cell, Cutaneous / drug therapy
  • Lymphoma, T-Cell, Cutaneous / virology
  • Male
  • Prednisone / therapeutic use
  • Receptors, Antigen, T-Cell, gamma-delta / metabolism
  • Skin Neoplasms / diagnosis*
  • Skin Neoplasms / drug therapy
  • Skin Neoplasms / virology
  • Vincristine / therapeutic use

Substances

  • Antineoplastic Agents
  • Receptors, Antigen, T-Cell, gamma-delta
  • Vincristine
  • Doxorubicin
  • Cyclophosphamide
  • Prednisone

Supplementary concepts

  • CHOP protocol