Defective neutrophil and lymphocyte function in leucocyte adhesion deficiency

Clin Exp Immunol. 1991 Aug;85(2):202-8. doi: 10.1111/j.1365-2249.1991.tb05705.x.

Abstract

We report a Chinese girl with the moderate phenotype of leucocyte adhesion deficiency (LAD), presenting with persistent omphalitis and recurrent soft tissue infections. She had subnormal adhesion-dependent neutrophil functions, such as chemotaxis and chemiluminescence response to a particulate stimulant (opsonised zymosan). Despite her adequate humoral response to documented herpes simplex virus type 1, parainfluenza type 2 and adenovirus infection in vivo, there was marked impairment in the generation of plaque-forming cells (PFC) driven by pokeweed mitogen (PWM) in vitro. IgM PFC were less severely affected than IgG and IgA PFC, probably because IgM production is less dependent on T cell help than IgA and IgG production. The patient's B cells and accessory cells had reduced function compared with the control subsets, while helper function of her CD4+ cells was virtually absent in the PWM-driven PFC assay. She also had marked defect in natural killer cell activity. The proliferation of her lymphocytes was normal to several plant lectins, including phytohaemagglutinin, concanavalin A and PWM, but markedly defective to OKT3.

Publication types

  • Case Reports

MeSH terms

  • Antigens, CD / deficiency
  • CD18 Antigens
  • Cell Adhesion
  • Child, Preschool
  • Female
  • Flow Cytometry
  • Fluorescent Antibody Technique
  • Humans
  • Killer Cells, Natural / immunology
  • Leukocyte-Adhesion Deficiency Syndrome*
  • Lymphocytes / immunology*
  • Macrophage-1 Antigen / deficiency
  • Neutrophils / immunology*

Substances

  • Antigens, CD
  • CD18 Antigens
  • Macrophage-1 Antigen