Enteropathy-type T-cell lymphoma expressing NK-cell intraepithelial lymphocyte (NK-IEL) phenotype

Leuk Lymphoma. 2004 Jul;45(7):1471-4. doi: 10.1080/10428190310001653709.

Abstract

Enteropathy-type T-cell lymphoma (ETL) is an intraepithelial T-lymphocyte (T-IEL) tumor. The tumor cells are usually CD3+, CD4-, CD8+, and contain cytotoxic granule associated proteins. We report on a CD3-negative CD56-positive enteropathy-associated lymphoma (ETL). This is the first case report of CD3-negative, CD56-positive, CD94-negative, and CD161-positive ETL. ETL cells originate from intraepithelial T-lymphocytes of the intestine. CD3-negative intraepithelial lymphocytes are known as natural killer (NK)-IELs. The phenotype of NK-IELs is also CD3-negative, CD56-positive, CD94-negative, and CD161-positive, while most normal NK cells express CD56 and CD94. CD3-negative lymphoma cells in this report also expressed CD56 and CD161, but not CD94. Because Southern blotting analysis showed a rearrangement of T-cell receptor (TCR) Cbeta in this case, the tumor is classified as an ETL. Based on the findings, NK-IELs may originate from T-cells, not NK-cells.

Publication types

  • Case Reports

MeSH terms

  • Antineoplastic Combined Chemotherapy Protocols / administration & dosage
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • CD3 Complex / analysis
  • CD56 Antigen / analysis
  • Cyclophosphamide / administration & dosage
  • Doxorubicin / administration & dosage
  • Endoscopy, Gastrointestinal
  • Fatal Outcome
  • Gastrointestinal Neoplasms / complications*
  • Gastrointestinal Neoplasms / diagnosis
  • Gastrointestinal Neoplasms / drug therapy
  • Gastrointestinal Neoplasms / pathology
  • Humans
  • Immunophenotyping
  • Intestinal Perforation / etiology
  • Killer Cells, Natural / chemistry
  • Killer Cells, Natural / pathology*
  • Leukocyte Common Antigens / analysis
  • Lymphocytes, Tumor-Infiltrating
  • Lymphoma, T-Cell / complications*
  • Lymphoma, T-Cell / diagnosis
  • Lymphoma, T-Cell / drug therapy
  • Lymphoma, T-Cell / pathology
  • Male
  • Middle Aged
  • Neoplastic Stem Cells / chemistry
  • Peritonitis / etiology
  • Phenotype
  • Prednisone / administration & dosage
  • Protein-Losing Enteropathies / etiology*
  • Vincristine / administration & dosage

Substances

  • CD3 Complex
  • CD56 Antigen
  • Vincristine
  • Doxorubicin
  • Cyclophosphamide
  • Leukocyte Common Antigens
  • Prednisone

Supplementary concepts

  • CHOP protocol