Primary immunodeficiencies: 2009 update - 12/08/11
International Union of Immunological Societies Expert Committee on Primary Immunodeficiencies
Luigi D. Notarangelo, MD a, ⁎
: Cochair, Alain Fischer, MD b : Cochair, Raif S. Geha, MD a, ⁎ : Cochair, Jean-Laurent Casanova, MD c, Helen Chapel, MD d, Mary Ellen Conley, MD e, Charlotte Cunningham-Rundles, MD, PhD f, Amos Etzioni, MD g, Lennart Hammartröm, MD h, Shigeaki Nonoyama, MD i, Hans D. Ochs, MD j, Jennifer Puck, MD k, Chaim Roifman, MD l, Reinhard Seger, MD m, Josiah Wedgwood, MD, PhD n
Reprint requests: Luigi D. Notarangelo, MD, or Raif S. Geha, MD, Division of Immunology, Children’s Hospital, One Blackfan Circle, Boston, MA 02115.Abstract |
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.
Le texte complet de cet article est disponible en PDF.Key words : Primary immunodeficiencies, T cells, B cells, severe combined immunodeficiency, predominantly antibody deficiencies, DNA repair defects, phagocytes, complement, immune dysregulation syndromes, innate immunity, autoinflammatory disorders
Abbreviations used : ADA, PID, SCID
| The Dublin meeting was supported by the Jeffrey Modell Foundation and by National Institute of Allergy and Infectious Diseases grant R13-AI-066891. Preparation of this report was supported by National Institutes of Health grant AI-35714 to R.S.G. and L.D.N. |
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| Disclosure of potential conflict of interest: J.-L. Casanova has consulted for Centocor. H. Chapel has received research support from Baxter Healthcare, Talecris, and Biotest. M. E. Conley has received research support from the National Institutes of Health. C. Cunningham-Rundles has received research support from Baxter Corp. A. Fischer has contracted for INSERM, the European Community, and the French National Research Agency. R. S. Geha has received research support from the National Institutes of Health and the March of Dimes. L. Hammartröm has received research support from the National Institutes of Health, the European Community, and the Swedish Research Council. H. D. Ochs is on advisory boards for Baxter and CSL Behring and has received research support from the Jeffrey Modell Foundation, the National Institutes of Health/National Institute of Allergy and Infectious Diseases, and Flebogamma. J. Puck has received research support from the National Institutes of Health, the Jeffrey Modell Foundation, and Baxter; is on committees for USID Net and the Immune Deficiency Foundation; and is a board member of the Immune Tolerance Institute. The rest of the authors have declared that they have no conflict of interest. |
Vol 124 - N° 6
P. 1161-1178 - décembre 2009 Retour au numéro
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