Primary immunodeficiencies: 2009 update.
More than 50 years after Ogdeon Bruton's discovery of congenital agammaglobulinemia, human primary immunodeficiencies (PIDs) continue to unravel novel molecular and cellular mechanisms that govern development and function of the human immune system. This report provides the updated classification of PIDs that has been compiled by the International Union of Immunological Societies Expert Committee on Primary Immunodeficiencies after its biannual meeting in Dublin, Ireland, in June 2009. Since the appearance of the last classification in 2007, novel forms of PID have been discovered, and additional pathophysiology mechanisms that account for PID in human beings have been unraveled. Careful analysis and prompt recognition of these disorders is essential to prompt effective forms of treatment and thus to improve survival and quality of life in patients affected with PIDs.
Division of Immunology, Children's Hospital Boston and Department of Pediatrics, Harvard Medical School, Boston, MA 02115, USA. luigi.notarangelo@childrens.harvard.edu
Notarangelo
Luigi D
LD
Fischer
Alain
A
Geha
Raif S
RS
Casanova
Jean-Laurent
JL
Chapel
Helen
H
Conley
Mary Ellen
ME
Cunningham-Rundles
Charlotte
C
Etzioni
Amos
A
Hammartröm
Lennart
L
Nonoyama
Shigeaki
S
Ochs
Hans D
HD
Puck
Jennifer
J
Roifman
Chaim
C
Seger
Reinhard
R
Wedgwood
Josiah
J
D015551:Autoimmunity / Q000276:immunology; D001402:B-Lymphocytes / Q000276:immunology / Q000378:metabolism; D003165:Complement System Proteins / Q000276:immunology* / Q000378:metabolism; D016207:Cytokines / Q000276:immunology / Q000378:metabolism; D006801:Humans; D007113:Immunity, Innate; D007136:Immunoglobulins / Q000097:blood; D007153:Immunologic Deficiency Syndromes / Q000145:classification* / Q000276:immunology* / Q000628:therapy; D007694:Killer Cells, Natural / Q000276:immunology / Q000378:metabolism; D013601:T-Lymphocytes / Q000276:immunology / Q000378:metabolism
D016207:Cytokines; D007136:Immunoglobulins; D003165:Complement System Proteins