Papillon-Lefèvre syndrome. Analysis of peripheral blood lymphocyte subsets

J Clin Periodontol. 1996 Sep;23(9):823-5. doi: 10.1111/j.1600-051x.1996.tb00618.x.

Abstract

We have studied the peripheral blood lymphocyte populations in our 6 patients (2 female and 4 male) with a mean age of 11.19 with Papillon-Lèfevre Syndrome (PLS) using adequate monoclonal antibodies and double coloured flow cytometry. Total B, T, CD4, CD8, CD29, CD45RA, NK, HLA-DR cells were studied. Total B, T, CD4 and CD8 lymphocytes were within normal limits. We have observed an increase in the CD29 lymphocytes and NK cells and a decrease in CD45RA lymphocytes. We think that these findings are important in explaining B lymphocyte activation and in the pathogenesis of the PLS.

MeSH terms

  • Alveolar Bone Loss / pathology
  • Antibodies, Monoclonal
  • B-Lymphocytes / pathology
  • CD4-Positive T-Lymphocytes / pathology
  • CD8-Positive T-Lymphocytes / pathology
  • Case-Control Studies
  • Child
  • Female
  • Flow Cytometry
  • HLA-DR Antigens / analysis
  • Humans
  • Integrin beta1 / analysis
  • Killer Cells, Natural / pathology
  • Leukocyte Common Antigens / analysis
  • Lymphocyte Activation
  • Lymphocyte Subsets / classification*
  • Lymphocyte Subsets / pathology
  • Male
  • Papillon-Lefevre Disease / blood*
  • Papillon-Lefevre Disease / pathology
  • Periodontal Attachment Loss / pathology
  • T-Lymphocytes / pathology
  • Tooth Migration / pathology
  • Tooth Mobility / pathology

Substances

  • Antibodies, Monoclonal
  • HLA-DR Antigens
  • Integrin beta1
  • Leukocyte Common Antigens