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, Irini Sereti

Research output: Contribution to journalArticlepeer-review

Abstract

Diopathic CD4+ lymphocytopenia (ICL) is a rare non-HI V-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/mm3 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/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 = .003 and .02, respectively). This trial is registered at http://clinicaltrials. govas #NCT00001319.

Original languageEnglish (US)
Pages (from-to)287-294
Number of pages8
JournalBlood
Volume112
Issue number2
DOIs
StatePublished - Jul 15 2008

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

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