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Blood, 15 July 2008, Vol. 112, No. 2, pp. 277-286. Prepublished online as a Blood First Edition Paper on March 4, 2008; DOI 10.1182/blood-2007-11-124545. This Article Full Text (PDF) Supplemental Figures All Versions of this Article: blood-2007-11-124545v1 112/2/277    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Chapel, H. Articles by Hammarstrom, L. Search for Related Content PubMed PubMed Citation Articles by Chapel, H. Articles by Hammarstrom, L. Related Collections Related Article in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted November 29, 2007 Accepted February 26, 2008 Common variable immunodeficiency disorders: division into distinct clinical phenotypes Helen Chapel*, Mary Lucas, Martin Lee, Janne Bjorkander, David Webster, Bodo Grimbacher, Clare Fieschi, Vojtech Thon, Mohammad R. Abedi, and Lennart Hammarstrom Department of Clinical Immunology, Nuffield Department of Medicine, University of Oxford, Oxford, United Kingdom Department of Biostatistics, University of California at Los Angeles, Los Angeles, CA, United States Department of Immunology and Allergy, Goteborg, Sweden Department of Immunology, Royal Free Hospital, London, United Kingdom Department of Immunology and Rheumatology, Freiburg, Germany Department of Medicine, Paris, France Department of Clinical Immunology, Masaryk University, Brno, Czech Republic Department of Transfusion Medicine, Orebro University Hospital, Orebro, Sweden Department of Clinical Immunology, Karolinska Institute at KUS Huddinge, Stockholm, Sweden * Corresponding author; email: helen.chapel{at}ndm.ox.ac.uk. The European Common Variable Immunodeficiency Disorders registry was started in 1996, in order to define distinct clinical phenotypes and determine overlap within individual patients. Seven centres contributed patient data resulting in the largest cohort yet reported. Patients (334), validated for the diagnosis, were followed for an average of 25.6 years [9,461 patient-years]. Data was used to define five distinct clinical phenotypes: no complications, autoimmunity, polyclonal lymphocytic infiltration, enteropathy and lymphoid malignancy. Eighty-three percent of patients had only one of these phenotypes. Analysis of mortality showed a considerable reduction in the last 15 years and that different phenotypes were associated with different survival times. Types of complications and clinical phenotypes varied significantly between countries, indicating the need for large, international registries. Ages at onset of symptoms and diagnosis were shown to have a Gaussian distribution, but were not useful predictors of phenotype. The only clinical predictor was polyclonal lymphocytic infiltration, which was associated with a five-fold increased risk of lymphoid malignancy. There was widespread variation in the levels of serum immunoglobulin isotypes as well as in the percentages and absolute numbers of B cells, confirming the heterogeneity of these conditions. Higher serum IgM and lower circulating CD8 proportions were found to be predictive markers for polyclonal lymphocytic infiltration and autoimmunity, respectively. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? Related Article in Blood Online: T-cell homeostasis: the dark(ened) side of common variable immunodeficiency Antonello Giovannetti, Marina Pierdominici, and Fernando Aiuti Blood 2008 112: 446. [Full Text] [PDF]

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