Central nervous system toxoplasmosis with an increased proportion of circulating gamma delta T cells in a patient with hyper-IgM syndrome

J Clin Immunol. 1998 Jul;18(4):283-90. doi: 10.1023/a:1027337923709.

Abstract

Hyper-IgM syndrome represents a diverse group of immunodeficiencies characterized by normal or high serum IgM concentrations with decreased or absent IgG, IgA, and IgE. The X-linked form of hyper-IgM syndrome is caused by mutations in the CD40 ligand gene, preventing its expression on activated T cells. The CD40 ligand--CD40 interaction is critical for effective isotype switching and for initiating antigen-specific Tf cell responses. In addition to recurrent pyogenic infections, patients with the CD40L defect also have opportunistic infections. An increased proportion of circulating gamma-delta T cells, shown to be important early during primary infections, has been demonstrated in numerous infectious diseases including toxoplasmosis. Here, we report a patient with hyper-IgM syndrome and CNS toxoplasmosis, who showed a marked increase in gamma-delta T cells in his peripheral blood and who has responded well to treatment of his toxoplasmosis and to high-dose immunoglobulin replacement therapy.

Publication types

  • Case Reports

MeSH terms

  • Aging
  • Animals
  • Brain / pathology
  • CD40 Antigens / metabolism
  • Central Nervous System Infections / complications*
  • Central Nervous System Infections / parasitology
  • Child
  • Flow Cytometry
  • Humans
  • Hypergammaglobulinemia / complications*
  • Immunoglobulin M / biosynthesis*
  • Immunoglobulins / blood
  • Immunologic Deficiency Syndromes / genetics
  • Lymphocyte Activation
  • Magnetic Resonance Imaging
  • Male
  • T-Lymphocytes / immunology*
  • Toxoplasma / isolation & purification*
  • Toxoplasmosis / complications*
  • Toxoplasmosis / pathology

Substances

  • CD40 Antigens
  • Immunoglobulin M
  • Immunoglobulins