Most CD56+ intestinal lymphomas are CD8+CD5-T-cell lymphomas of monomorphic small to medium size histology

Am J Pathol. 1998 Nov;153(5):1483-90. doi: 10.1016/S0002-9440(10)65736-7.


The expression of the natural killer (NK) cell marker CD56 has been reported to occur in NK cell lymphomas/leukemias and a small group of peripheral T-cell lymphomas but has not been studied extensively in primary intestinal non-B-cell lymphomas. Normal human jejunal intraepithelial lymphocytes (IELs) are mainly T-cell receptor (TCR)-alphabeta+CD3+CD8+CD5low and include an approximately 15% fraction of CD56+ cells that could be the cells of origin for CD56+ intestinal T-cell lymphoma (ITL). To test this hypothesis, 70 cases diagnosed as ITL were immunophenotyped, and 15 CD56+ cases (21%) were identified. The majority of the CD56+ lymphomas was of monomorphic small to medium-sized histology, shared the common phenotype betaF1+/-CD3epsilon/cyt+CD8+CD4-CD5-CD57-TIA-1+ and had clonally rearranged TCR gamma-chain genes. In contrast, the CD56- lymphomas were mainly composed of pleomorphic medium and large cells or had a morphology most consistent with anaplastic large-cell lymphoma and were mostly CD8-. These findings suggest that the majority of CD56+ intestinal lymphomas are morphologically and phenotypically distinct T-cell lymphomas most likely derived from activated cytotoxic CD56+CD8+ IELs. Some overlapping histological and clinical features between CD56+ and CD56- ITLs indicate that the former belong to the clinicopathological entity of ITL. The consistent expression of cytotoxic-granule-associated proteins introduces ITL (both CD56+ and CD56-) into the growing family of usually aggressive extranodal lymphomas of cytotoxic T-cell and NK-cell derivation. In contrast to putative NK-cell lymphoma of the sinonasal region, intestinal NK-cell lymphoma seems to be very rare.

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Biomarkers, Tumor / analysis
  • CD5 Antigens / analysis*
  • CD56 Antigen / analysis*
  • CD8 Antigens / analysis*
  • Clone Cells / chemistry
  • Female
  • Humans
  • Immunophenotyping
  • Intestinal Neoplasms / chemistry
  • Intestinal Neoplasms / immunology*
  • Intestinal Neoplasms / pathology
  • Lymphoma, T-Cell / chemistry
  • Lymphoma, T-Cell / immunology*
  • Lymphoma, T-Cell / pathology
  • Male
  • Middle Aged
  • Receptors, Antigen, T-Cell, gamma-delta / immunology


  • Biomarkers, Tumor
  • CD5 Antigens
  • CD56 Antigen
  • CD8 Antigens
  • Receptors, Antigen, T-Cell, gamma-delta