Central nervous system toxoplasmosis with an increased proportion of circulating gamma delta T cells in a patient with hyper-IgM syndrome.
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.
Leiva
L E
LE
Department of Pediatrics, Louisiana State University Medical Center, New Orleans 70112-2822, USA.
Junprasert
J
J
Hollenbaugh
D
D
Sorensen
R U
RU
D000375:Aging; D000818:Animals; D001921:Brain / Q000473:pathology; D019013:CD40 Antigens / Q000378:metabolism; D002494:Central Nervous System Infections / Q000150:complications* / Q000469:parasitology; D002648:Child; D005434:Flow Cytometry; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications*; D007075:Immunoglobulin M / Q000096:biosynthesis*; D007136:Immunoglobulins / Q000097:blood; D007153:Immunologic Deficiency Syndromes / Q000235:genetics; D008213:Lymphocyte Activation; D008279:Magnetic Resonance Imaging; D008297:Male; D013601:T-Lymphocytes / Q000276:immunology*; D014122:Toxoplasma / Q000302:isolation & purification*; D014123:Toxoplasmosis / Q000150:complications* / Q000473:pathology
D019013:CD40 Antigens; D007075:Immunoglobulin M; D007136:Immunoglobulins