FREE NEJM E-TOC    HOME   |   SUBSCRIBE   |   CURRENT ISSUE   |   PAST ISSUES   |   COLLECTIONS   |   Search Term  Advanced Search

Sign in | Get NEJM's E-Mail Table of Contents — Free | Subscribe

Previous Volume 328:393-398 February 11, 1993 Number 6 Next

	Full Text Add to Personal Archive Add to Citation Manager Notify a Friend E-mail When Cited PubMed Citation

ABSTRACT

Background and 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.

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.

Full Text of this Article

This article has been cited by other articles:

• Augusto, E., Raguenaud, M.-E., Kim, C., Mony, M., Isaakidis, P. (2009). Idiopathic CD4+ T-lymphocytopenia with cryptococcal meningitis: first case report from Cambodia. Trop Doct 39: 176-177 [Abstract] [Full Text]  
• 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. (2008). Idiopathic CD4+ lymphocytopenia: natural history and prognostic factors. Blood 112: 287-294 [Abstract] [Full Text]  
• Niino, D., Tsukasaki, K., Torii, K., Imanishi, D., Tsuchiya, T., Onimaru, Y., Tsushima, H., Yoshida, S., Yamada, Y., Kamihira, S., Tomonaga, M. (2008). Human Herpes virus 8-negative primary effusion lymphoma with BCL6 rearrangement in a patient with idiopathic CD4 positive T-lymphocytopenia. haematol 93: e21-e23 [Abstract] [Full Text]  
• Malaspina, A., Moir, S., Chaitt, D. G., Rehm, C. A., Kottilil, S., Falloon, J., Fauci, A. S. (2007). Idiopathic CD4+ T lymphocytopenia is associated with increases in immature/transitional B cells and serum levels of IL-7. Blood 109: 2086-2088 [Abstract] [Full Text]  
• Bharadwaj, J. S., Goulet, C. J., Tonnerre, C. (2005). The Unturned Stone. NEJM 352: 2140-2141 [Full Text]  
• Hutchinson, P., Chadban, S. J., Atkins, R. C., Holdsworth, S. R. (2003). Laboratory assessment of immune function in renal transplant patients. Nephrol Dial Transplant 18: 983-989 [Abstract] [Full Text]  
• Kilic, S S., Bostan, O, Cil, E (2002). Takayasu arteritis. Ann Rheum Dis 61: 92-93 [Full Text]  
• Hubert, P., Bergeron, F., Ferreira, V., Seligmann, M., Oksenhendler, E., Debre, P., Autran, B. (2000). Defective p56Lck activity in T cells from an adult patient with idiopathic CD4+ lymphocytopenia. Int Immunol 12: 449-457 [Abstract] [Full Text]  
• Walzer, P. D. (1999). Immunological Features of Pneumocystis carinii Infection in Humans. CVI 6: 149-155 [Full Text]  
• Zollner, T. M., Stracke, S., Neumeister, B., Manfras, B., Boehncke, W.-H., Boehm, B. O., Marre, R., Sterry, W. (1996). Idiopathic CD4+ T Lymphocytopenia Presenting as Mycetoma in a Patient With a Mutation in the Cystic Fibrosis Transmembrane Regulator Gene. Arch Dermatol 132: 1247-1249 [Abstract]  
• Sleasman, J.W. (1996). The Association Between Immunodeficiency and the Development of Autoimmune Disease. ADR 10: 57-61 [Abstract]  
• Holland, S. M., Eisenstein, E. M., Kuhns, D. B., Turner, M. L., Fleisher, T. A., Strober, W., Gallin, J. I. (1994). Treatment of Refractory Disseminated Nontuberculous Mycobacterial Infection With Interferon Gamma: A Preliminary Report. NEJM 330: 1348-1355 [Abstract] [Full Text]  
• DeHovitz, J. A., Feldman, J., Landesman, S. (1993). Idiopathic CD4+ T-Lymphocytopenia. NEJM 329: 1045-1046 [Full Text]  
• Goodrich, A. L., Tigelaar, R. E., Watsky, K. L., Heald, P. W. (1993). Idiopathic CD4+ Lymphocyte Deficiency: Report of an Unusual Case Associated With Atopic Dermatitis and Allergic Contact Dermatitis and Review of the Literature. Arch Dermatol 129: 876-878 [Abstract]  
• Sheppard, H., Winkelstein, W., Lang, W., Charlebois, E., Heymann, D. L., Belsey, E., Esparza, J. G., Laurence, J., Corboy, J. R., Stevens, S. R., Griffiths, T. W., Cooper, K. D., Smith, D. K., Neal, J. J., Holmberg, S. D. (1993). CD4+ T-Lymphocytopenia without HIV Infection. NEJM 328: 1847-1850 [Full Text]  
• (1993). Putting the 'New' AIDS-Like Illness in Perspective. Journal Watch Dermatology 1993: 18-18 [Full Text]  
• (1993). PUTTING THE ""NEW"" AIDS-LIKE ILLNESS IN PERSPECTIVE. JWatch General 1993: 1-1 [Full Text]  
• Smith, D. K., Neal, J. J., Holmberg, S. D., The Centers for Disease Control Idiopathic CD4+ T-, (1993). Unexplained Opportunistic Infections and CD4+ T-Lymphocytopenia without HIV Infection -- An Investigation of Cases in the United States. NEJM 328: 373-379 [Abstract] [Full Text]  
• Fauci, A. S. (1993). CD4+ T-Lymphocytopenia without HIV Infection -- No Lights, No Camera, Just Facts. NEJM 328: 429-431 [Full Text]