title: Idiopathic CD4+ lymphocytopenia: natural history and prognostic factors.
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).
authors.affiliation: Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892, USA.
authors.forename: Dimitrios I
authors.initials: DI
authors.lastname: Zonios
authors.forename: Judith
authors.initials: J
authors.lastname: Falloon
authors.forename: John E
authors.initials: JE
authors.lastname: Bennett
authors.forename: Pamela A
authors.initials: PA
authors.lastname: Shaw
authors.forename: Doreen
authors.initials: D
authors.lastname: Chaitt
authors.forename: Michael W
authors.initials: MW
authors.lastname: Baseler
authors.forename: Joseph W
authors.initials: JW
authors.lastname: Adelsberger
authors.forename: Julia A
authors.initials: JA
authors.lastname: Metcalf
authors.forename: Michael A
authors.initials: MA
authors.lastname: Polis
authors.forename: Stephen B
authors.initials: SB
authors.lastname: Kovacs
authors.forename: Stephen J
authors.initials: SJ
authors.lastname: Kovacs
authors.forename: Joseph A
authors.initials: JA
authors.lastname: Kovacs
authors.forename: Richard T
authors.initials: RT
authors.lastname: Davey
authors.forename: H Clifford
authors.initials: HC
authors.lastname: Lane
authors.forename: Henry
authors.initials: H
authors.lastname: Masur
authors.forename: Irini
authors.initials: I
authors.lastname: Sereti
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001327:Autoimmune Diseases; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008213:Lymphocyte Activation; D008231:Lymphopenia / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology* / Q000382:microbiology; D008297:Male; D008875:Middle Aged; D009894:Opportunistic Infections; D011379:Prognosis; D011446:Prospective Studies; D016019:Survival Analysis; D013601:T-Lymphocytes