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Blood, 15 July 2008, Vol. 112, No. 2, pp. 287-294. Prepublished online as a Blood First Edition Paper on May 2, 2008; DOI 10.1182/blood-2007-12-127878. This Article Full Text (PDF) All Versions of this Article: blood-2007-12-127878v1 112/2/287    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 HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Zonios, D. I. Articles by Sereti, I. Search for Related Content PubMed PubMed Citation Articles by Zonios, D. I. Articles by Sereti, I. Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted December 14, 2007 Accepted April 7, 2008 Idiopathic CD4+ lymphocytopenia: natural history and prognostic factors Dimitrios I. Zonios, Judith Falloon, John E. Bennett, Pamela A. Shaw, Doreen Chaitt, Michael W. Baseler, Joseph W. Adelsberger, Julia A. Metcalf, Michael A. Polis, Stephen J Kovacs, Joseph A. Kovacs, Richard T. Davey, H. Clifford Lane, Henry Masur, and Irini Sereti* Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, United States Laboratory of Immunoregulation, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, United States Biostatistics Research Branch, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, United States Applied and Developmental Research Support Program, Science Application International Corporation (SAIC), National Cancer Institute at Frederick, Frederick, MD, United States Critical Care Medicine Department, Warren G. Magnuson Clinical Center, National Institutes of Health, Bethesda, MD, United States * Corresponding author; email: isereti{at}mail.nih.gov. Idiopathic CD4+ lymphocytopenia (ICL) is a rare non-HIV related syndrome with unclear natural history and prognosis. This prospective natural history cohort study describes the clinical course, CD4 T lymphocyte kinetics, outcome and prognostic factors of ICL. Thirty nine patients (17 men, 22 women), 25 to 85 years old with ICL were evaluated between 1992 and 2006 and thirty six were followed for a median of 49.5 months. Cryptococcal and non-tuberculous mycobacterial infections were the major presenting opportunistic infections. Seven patients presented with no infection. In thirty two, CD4 T cell counts remained below 300/mm3 throughout the study period and in seven normalized after an average of 31 months. Overall, fifteen (41.6%) developed an opportunistic infection in follow up, five (13.8%) of which were "AIDS defining clinical conditions", and four (11.1%) developed autoimmune diseases. Seven patients died, four from ICL related opportunistic infections within 42 months after diagnosis. Immunologic analyses revealed increased activation and turnover in CD4 but not CD8 T lymphocytes. CD8 T lymphocytopenia (<180/mm3) and the degree of CD4 T cell activation (measured by HLA-DR expression) at presentation were associated with adverse outcome (Opportunistic infection related death) (p=0.003 and 0.02 respectively). This trial is registered at http://clinicaltrials.gov as NCT00001319. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: J. Thoden, N. Venhoff, M. Daskalakis, A. Schmitt-Graeff, R. Drager, M. Schlesier, K. Warnatz, and H.-H. Peter Disseminated tuberculosis in a patient with idiopathic CD4+ lymphocytopenia Rheumatology, October 1, 2009; 48(10): 1329 - 1330. [Full Text] [PDF]

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