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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 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 Citation Map 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 HighWire 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 Immunobiology Free Research Articles Clinical Trials and Observations Related Article in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  CLINICAL TRIALS AND OBSERVATIONS Common variable immunodeficiency disorders: division into distinct clinical phenotypes Helen Chapel1, Mary Lucas1, Martin Lee2, Janne Bjorkander3, David Webster4, Bodo Grimbacher4,5, Claire Fieschi6, Vojtech Thon7, Mohammad R. Abedi8,9, and Lennart Hammarstrom9 1 Department of Clinical Immunology, Nuffield Department of Medicine, University of Oxford, Oxford, United Kingdom; 2 Department of Biostatistics, University of California at Los Angeles; 3 Department of Immunology and Allergy, Sahlgrenska University Hospital, Gothenberg, Sweden; 4 Department of Immunology, Royal Free Hospital, London, United Kingdom; 5 Department of Immunology and Rheumatology, University of Freiburg, Freiburg, Germany; 6 Department of Medicine, Saint-Louis Hospital, Paris, France; 7 Department of Clinical Immunology, Masaryk University, Brno, Czech Republic; 8 Department of Transfusion Medicine, Örebro University Hospital, Örebro, Sweden; and 9 Department of Clinical Immunology, Karolinska Institute at KUS Huddinge, Stockholm, Sweden The European Common Variable Immunodeficiency Disorders registry was started in 1996 to define distinct clinical phenotypes and determine overlap within individual patients. A total of 7 centers 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 (9461 patient-years). Data were used to define 5 distinct clinical phenotypes: no complications, autoimmunity, polyclonal lymphocytic infiltration, enteropathy, and lymphoid malignancy. A total of 83% 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 5-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] This article has been cited by other articles: P. Wood and D. Peckham Investigating recurrent respiratory infections in primary care BMJ, November 12, 2009; 339(nov12_2): b4118 - b4118. [Full Text] S. Borte, Q. Pan-Hammarstrom, C. Liu, U. Sack, M. Borte, U. Wagner, D. Graf, and L. Hammarstrom Interleukin-21 restores immunoglobulin production ex vivo in patients with common variable immunodeficiency and selective IgA deficiency Blood, November 5, 2009; 114(19): 4089 - 4098. [Abstract] [Full Text] [PDF] A. J. Fried and F. A. Bonilla Pathogenesis, Diagnosis, and Management of Primary Antibody Deficiencies and Infections Clin. Microbiol. Rev., July 1, 2009; 22(3): 396 - 414. [Abstract] [Full Text] [PDF] U. Salzer, C. Bacchelli, S. Buckridge, Q. Pan-Hammarstrom, S. Jennings, V. Lougaris, A. Bergbreiter, T. Hagena, J. Birmelin, A. Plebani, et al. Relevance of biallelic versus monoallelic TNFRSF13B mutations in distinguishing disease-causing from risk-increasing TNFRSF13B variants in antibody deficiency syndromes Blood, February 26, 2009; 113(9): 1967 - 1976. [Abstract] [Full Text] [PDF] A. Giovannetti, M. Pierdominici, and F. Aiuti T-cell homeostasis: the dark(ened) side of common variable immunodeficiency Blood, July 15, 2008; 112(2): 446 - 446. [Full Text] [PDF]

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