Rare provisional entity: primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma in a young woman

J Clin Pathol. 2008 Jun;61(6):770-2. doi: 10.1136/jcp.2007.054585.

Abstract

Primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma is a rare and provisional entity, characterised by cutaneous involvement and aggressive clinical behaviour. The case is here presented of a young woman with concurrent cutaneous and systemic involvement. Despite multi-agent chemotherapy, only partial remission could be achieved, and the patient died from therapy-resistant respiratory and circulatory failure. This case report is intended to add to the data collected on this rare entity, with only about 20 cases as yet described.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Antigens, CD / analysis
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Biomarkers / analysis
  • CD8-Positive T-Lymphocytes / immunology
  • Cyclophosphamide / therapeutic use
  • Dexamethasone / therapeutic use
  • Doxorubicin / therapeutic use
  • Female
  • Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor
  • Humans
  • Immunophenotyping
  • Karyotyping
  • Lymphoma, T-Cell, Cutaneous / diagnosis*
  • Lymphoma, T-Cell, Cutaneous / genetics
  • Lymphoma, T-Cell, Cutaneous / immunology
  • Skin Neoplasms / diagnosis*
  • Skin Neoplasms / genetics
  • Skin Neoplasms / immunology
  • Translocation, Genetic
  • Treatment Failure
  • Vincristine / therapeutic use

Substances

  • Antigens, CD
  • Biomarkers
  • Vincristine
  • Dexamethasone
  • Doxorubicin
  • Cyclophosphamide

Supplementary concepts

  • CVAD protocol