title: Idiopathic CD4+ T-lymphocytopenia--four patients with opportunistic infections and no evidence of HIV infection.
abstract: METHODS
  We describe four patients without major risk factors for human immunodeficiency virus (HIV) infection, each of whom presented with severe opportunistic infections and was found to have idiopathic CD4+ T-lymphocytopenia. We performed assays to detect the presence of retroviruses and undertook immunophenotyping of subgroups of peripheral-blood lymphocytes.
  
  
  RESULTS
  The opportunistic infections at presentation included Pneumocystis carinii pneumonia, cryptococcal meningitis (two patients, one with concurrent pulmonary tuberculosis), and histoplasma-induced brain abscess. During 10 to 68 months of observation, none of the four patients had evidence of infection with HIV type 1 or 2 or human T-cell lymphotropic virus type I or II on the basis of epidemiologic, serologic, or polymerase-chain-reaction studies or culture, nor was there any detectable reverse transcriptase activity. Although all the patients had severe, persistent CD4+ T-lymphocytopenia (range, 12 to 293 cells per cubic millimeter), the CD4+ cell count progressively declined in only one and was accompanied by multiple opportunistic infections. All four patients had significantly reduced numbers of circulating CD8+ T cells, natural killer cells, or B cells (or all three).
  
  
  CONCLUSIONS
  These four patients had idiopathic CD4+ T-lymphocytopenia with opportunistic infections but no evidence of HIV infection. Instead of the progressive, selective depletion of CD4+ T cells characteristic of HIV infection, some patients with idiopathic immunodeficiency have stable CD4+ cell counts accompanied by reductions in the levels of several other lymphocyte subgroups.
authors.affiliation: Evans Memorial Department of Clinical Research, Boston City Hospital, MA 02118.
authors.forename: R A
authors.initials: RA
authors.lastname: Duncan
authors.forename: C F
authors.initials: CF
authors.lastname: von Reyn
authors.forename: G M
authors.initials: GM
authors.lastname: Alliegro
authors.forename: Z
authors.initials: Z
authors.lastname: Toossi
authors.forename: A M
authors.initials: AM
authors.lastname: Sugar
authors.forename: S M
authors.initials: SM
authors.lastname: Levitz
mesh_terms: D000328:Adult; D000368:Aged; D001402:B-Lymphocytes; D015496:CD4-Positive T-Lymphocytes; D005260:Female; D005434:Flow Cytometry; D006660:Histoplasmosis / Q000150:complications; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications; D007694:Killer Cells, Natural; D007958:Leukocyte Count; D008231:Lymphopenia / Q000209:etiology*; D008297:Male; D016919:Meningitis, Cryptococcal / Q000150:complications; D009894:Opportunistic Infections / Q000150:complications*; D011020:Pneumonia, Pneumocystis / Q000150:complications; D012190:Retroviridae / Q000276:immunology / Q000302:isolation & purification; D012192:Retroviridae Infections / Q000175:diagnosis; D014397:Tuberculosis, Pulmonary / Q000150:complications