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Idiopathic CD4+ lymphocytopenia: natural history and prognostic factors

  1. Author:
    Zonios, D. I.
    Falloon, J.
    Bennett, J. E.
    Shaw, P. A.
    Chaitt, D.
    Baseler, M. W.
    Adelsberger, J. W.
    Metcalf, J. A.
    Polis, M. A.
    Kovacs, S. J.
    Kovacs, J. A.
    Davey, R. T.
    Lane, H. C.
    Masur, H.
    Sereti, I.
  2. Author Address

    Zonios, Dimitrios I.; Bennett, John E.] NIAID, Natl Inst Hlth, Lab Clin Infect Dis, Bethesda, MD 20892 USA. [Falloon, Judith, Chaitt, Doreen, Metcalf, Julia A.; Polis, Michael A.; Kovacs, Stephen J.; Davey, Richard T.; Lane, H. Clifford, Sereti, Irini] NIAID, Natl Inst Hlth, Immunoregulat Lab, Bethesda, MD 20892 USA. [Shaw, Pamela A.] NIAID, Natl Inst Hlth, Biostat Res Branch, Bethesda, MD 20892 USA. [Baseler, Michael W.; Adelsberger, Joseph W.] Sci Applicat Int Corp, NCI, Appl & Dev Res Support Program, Frederick, MD USA. [Masur, Henry] Natl Inst Hlth, Dept Crit Care Med, Warren G Magnuson Clin Ctr, Bethesda, MD USA.
    1. Year: 2008
  1. Journal: Blood
    1. 112
    2. 2
    3. Pages: 287-294
  2. Type of Article: Article
  1. Abstract:

    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 36 were followed for a median of 49.5 months. Cryptococcal and nontuberculous mycobacterial infections were the major presenting opportunistic infections. Seven patients presented with no infection. In 32, CD4 T-cell counts remained less than 300/mm(3) throughout the study period and in 7 normalized after an average of 31 months. Overall, 15 (41.6%) developed an opportunistic infection in follow-up, 5 (13.8%) of which were "AIDS-defining clinical conditions," and 4 (11.1 %) developed autoimmune diseases. Seven patients died, 4 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/mm(3)) 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 = .003 and .02, respectively). This trial is registered at http://clinicaltrials. gov as #NCT00001319.

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External Sources

  1. PMID: 18456875

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