ISSN:
1573-2592
Keywords:
Hyper-IgM syndrome
;
CNS toxoplasmosis
;
γδ T cells
;
CD40 ligand
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
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 T cell responses. In addition to recurrent pyogenic infections, patients with the CD40L defect also have opportunistic infections. An increased proportion of circulating γδ 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 γδ T cells in his peripheral blood and who has responded well to treatment of his toxoplasmosis and to high-dose immunoglobulin replacement therapy.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1023/A:1027337923709
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