Abnormal proliferation of CD4- CD8+ gammadelta+ T cells with chromosome 6 anomaly: role of Fas ligand expression in spontaneous regression of the cells

Am J Hematol. 1999 Apr;60(4):305-8. doi: 10.1002/(sici)1096-8652(199904)60:4<305::aid-ajh9>3.0.co;2-6.

Abstract

We report a case of granular lymphocyte proliferative disorder accompanied with hemolytic anemia and neutropenia. Phenotypes of the cells were T cell receptor gammadelta+ CD3+ CD4- CD8+ CD16+ CD56- CD57-. Southern blot analysis of T cell receptor beta and gamma chains demonstrated rearranged bands in both. Chromosomal analysis after IL-2 stimulation showed deletion of chromosome 6. Sorted gammadelta+ T cells showed an increase in Fas ligand expression compared with the levels in sorted alphabeta+ T cells. The expression of Fas ligand on these gammadelta+ T cells increased after IL-2 stimulation. The patient's anemia improved along with a decrease in granular lymphocyte count and disappearance of the abnormal karyotype without treatment. The expression of Fas ligand may be involved in spontaneous regression of granular lymphocyte proliferation with hemolytic anemia.

Publication types

  • Case Reports

MeSH terms

  • Anemia, Hemolytic / genetics
  • Blotting, Southern
  • CD4 Antigens / analysis
  • CD8 Antigens / analysis
  • Chromosomes, Human, Pair 6*
  • Fas Ligand Protein
  • Gene Deletion*
  • Humans
  • Immunophenotyping
  • Interleukin-2 / pharmacology
  • Karyotyping
  • Killer Cells, Natural / immunology
  • Lymphoproliferative Disorders / genetics*
  • Lymphoproliferative Disorders / pathology
  • Male
  • Membrane Glycoproteins / genetics*
  • Middle Aged
  • Neutropenia / genetics
  • Receptors, Antigen, T-Cell, gamma-delta / analysis
  • Remission, Spontaneous
  • T-Lymphocytes / immunology*
  • T-Lymphocytes / pathology
  • T-Lymphocytes, Cytotoxic / immunology

Substances

  • CD4 Antigens
  • CD8 Antigens
  • FASLG protein, human
  • Fas Ligand Protein
  • Interleukin-2
  • Membrane Glycoproteins
  • Receptors, Antigen, T-Cell, gamma-delta