Abnormal interleukin-7 function in common variable immunodeficiency.
Common variable immunodeficiency (CVID) is characterized by low levels of circulating immunoglobulins, leading to frequent infections, particularly of the respiratory tract. Frequently, T-cell abnormalities are observed. Interleukin-7 (IL-7) is involved in the homeostasis of lymphocytes, and may be elevated in lymphopenia. Mutations of genes related to IL-7 may lead to severe immunodeficiency disorders. We report elevated plasma levels of circulating IL-7 in a subgroup of CVID. These patients have increased numbers of circulating CD8+ T cells with decreased apoptosis and a predominance of CC chemokine receptor 7- (CCR7-) effector-memory T cells. Moreover, in some of these patients there is impaired response to IL-7 as assessed by in vitro proliferation and secretion of interferon gamma and transforming growth factor beta. These findings suggest novel pathogenic mechanisms and specific targets for further research in CVID.
Holm
Are Martin
AM
Section of Clinical Immunology and Infectious Diseases, Medical Center, Oslo, Norway. a.m.holm@klinmed.uio.no
Aukrust
Pål
P
Damås
Jan Kristian
JK
Müller
Fredrik
F
Halvorsen
Bente
B
Frøland
Stig S
SS
D000328:Adult; D017209:Apoptosis / Q000276:immunology; D018414:CD8-Positive T-Lymphocytes / Q000276:immunology / Q000378:metabolism; D002455:Cell Division / Q000276:immunology; D017074:Common Variable Immunodeficiency / Q000276:immunology* / Q000378:metabolism*; D005260:Female; D006801:Humans; D015851:Interleukin-7 / Q000097:blood* / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D015398:Signal Transduction / Q000276:immunology
D015851:Interleukin-7