CD3+, CD4-, CD8- large granular T-cell lymphoproliferative disorder

Am J Hematol. 1991 Jul;37(3):173-8. doi: 10.1002/ajh.2830370308.

Abstract

Large granular T-cell lymphoproliferative disorder (LGTLD) is a heterogeneous disorder covering a broad spectrum of diseases and requiring further subdivision. Most reported cases emphasized its suppressor phenotype (T gamma cell or CD8+), but we encountered two cases of CD3+, CD4-, CD8- LGTLD. Both cases had a benign clinical course and required no chemotherapy despite persistent lymphocytosis. This unique phenotype has been reported in a few cases of acute lymphoblastic leukemia expressing the T-cell receptor (TcR) gamma chain gene and is considered the counterpart of thymocytes at the intermediate stage between early precursors and mature thymocytes. Our case 1 provides further evidence that the CD3+, CD4-, CD8- phenotype, indeed, expresses the TcR gamma chain gene. However, the negative reaction to terminal deoxynucleotidyl transferase in our case 1 indicates that this phenotype represents proliferation of peripheral T-cells, in which about 2% bear the CD3+, CD4-, CD8- phenotype in the normal population. The selective use of CD3, CD4, CD8, HNK-1 monoclonal antibodies and of cytochemical stains (acid phosphatase and alpha-naphthyl butyrate esterase) for characterization of this disorder is discussed.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Antigens, Differentiation, T-Lymphocyte / analysis*
  • CD3 Complex
  • CD4 Antigens / analysis*
  • CD8 Antigens
  • Flow Cytometry
  • Gene Rearrangement, beta-Chain T-Cell Antigen Receptor
  • Humans
  • Immunophenotyping
  • Killer Cells, Natural / immunology*
  • Lymphoproliferative Disorders / immunology*
  • Male
  • Receptors, Antigen, T-Cell / analysis*

Substances

  • Antigens, Differentiation, T-Lymphocyte
  • CD3 Complex
  • CD4 Antigens
  • CD8 Antigens
  • Receptors, Antigen, T-Cell