Background and Methods We describe four patients without majorrisk factors for human immunodeficiency virus (HIV) infection,each of whom presented with severe opportunistic infectionsand was found to have idiopathic CD4+ T-lymphocytopenia. Weperformed assays to detect the presence of retroviruses andundertook immunophenotyping of subgroups of peripheral-bloodlymphocytes.
Results The opportunistic infections at presentation includedPneumocystis carinii pneumonia, cryptococcal meningitis (twopatients, one with concurrent pulmonary tuberculosis), and histoplasma-inducedbrain abscess. During 10 to 68 months of observation, none ofthe four patients had evidence of infection with HIV type 1or 2 or human T-cell lymphotropic virus type I or II on thebasis of epidemiologic, serologic, or polymerase-chain-reactionstudies or culture, nor was there any detectable reverse transcriptaseactivity. 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 wasaccompanied by multiple opportunistic infections. All four patientshad 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-lymphocytopeniawith opportunistic infections but no evidence of HIV infection.Instead of the progressive, selective depletion of CD4+ T cellscharacteristic of HIV infection, some patients with idiopathicimmunodeficiency have stable CD4+ cell counts accompanied byreductions in the levels of several other lymphocyte subgroups.
Source Information
From the Evans Memorial Department of Clinical Research and the Department of Medicine, Thorndike Memorial Laboratory and the Maxwell Finland Laboratory for Infectious Diseases, Boston City Hospital and University Hospital, Boston University School of Medicine, Boston (R.A.D., G.M.A., A.M.S., S.M.L.); the Infectious Disease Section, Department of Medicine, Dartmouth-Hitchcock Medical Center, Lebanon, N.H. (C.F.v.R.); and Cleveland Veterans Affairs Medical Center and Case Western Reserve University, Cleveland (Z.T.). Presented at the 32nd Interscience Conference on Antimicrobial Agents and Chemotherapy, Anaheim, Calif., October 11-14, 1992.
Address reprint requests to Dr. Duncan at Thorndike Bldg. 311, Boston City Hospital, 818 Harrison Ave., Boston, MA 02118.
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