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

Volume 328:373-379 February 11, 1993 Number 6 Next

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

ABSTRACT

Background The clinical and public health importance of recent reports of patients with CD4+ T-lymphocytopenia without human immunodeficiency virus (HIV) infection is unclear. We conducted investigations to determine the demographic, clinical, and immunologic features of patients with idiopathic CD4+ T-lymphocytopenia; whether the syndrome is epidemic or transmissible; and the possible causes.

Methods We reviewed 230,179 cases in the Centers for Disease Control and Prevention (CDC) AIDS Reporting System and performed interviews, medical-record reviews, and laboratory analyses of blood specimens from adults and adolescents who met the CDC case definition of idiopathic CD4+ T-lymphocytopenia (<300 CD4+ cells per cubic millimeter or a CD4+ cell count <20 percent of total T cells on two occasions and no evidence of infection on HIV testing), their sexual contacts, household contacts, and persons who had donated blood to them.

Results We interviewed 31 of the 47 patients identified with idiopathic CD4+ T-lymphocytopenia and 23 of their contacts. There were 29 male and 18 female patients, with a mean age of 43 years (range, 17 to 78); 39 were white, 4 were Asian, 2 were Hispanic, and 2 were black. Eighteen patients (38 percent) had one or more risk factors for HIV infection: seven had hemophilia, six had engaged in homosexual sex, six had received blood transfusions, and two had had heterosexual sex partners who were at risk for HIV infection. The other 29 patients (62 percent) had no identified risk factors for HIV infection. Nineteen persons (40 percent) had AIDS-defining illnesses (18 had opportunistic infections), 25 (53 percent) had conditions that were not AIDS-defining, and 3 (6 percent) were asymptomatic. We tested blood from 28 patients: 8 (29 percent) were found to have CD4+ T-lymphocyte counts of less than 300 cells per cubic millimeter, and 6 had CD8+ T-lymphocytopenia (<250 cells per cubic millimeter). Ten sex partners, three household contacts, and four children of the patients, as well as six persons who had donated blood to the patients, were immunologically and clinically normal.

Conclusions This investigation of patients with idiopathic CD4+ T-lymphocytopenia and unexplained opportunistic infections indicates that the disorder is rare and represents various clinical and immunologic states. The investigation of contacts revealed no evidence of a new transmissible agent that causes lymphocytopenia.

Source Information

The members of the task force are as follows: Epidemiology and Surveillance Working Group: Martha F. Rogers, M.D. (chair); Epidemiologic Investigations: Scott D. Holmberg, M.D., M.P.H. (coordinator), Thomas J. Spira, M.D., Dawn K. Smith, M.D., M.P.H., M.S., Pascale M. Wortley, M.D., M.P.H., Lisa A. Jackson, M.D. (Division of Bacterial and Mycobacterial Diseases, National Center for Infectious Diseases [NCID]), Judith R. Rudnick, M.D. (Hospital Infections Program, NCID), Judith Falloon, M.D., and Doreen G. Chaitt, R.N., M.P.H. (Laboratory of Immunoregulation, National Institute of Allergy and Infectious Diseases); Surveillance Operations: I. Celine Hanson, M.D., and John W. Ward, M.D. (coordinators), Joyce J. Neal, Ph.D., M.P.H., Kenneth A. Bell, Laurence Slutsker, M.D., M.P.H., and C. Rene Jones; Other Members: Harold W. Jaffe, M.D., Mark N. Lobato, M.D., Kenneth G. Castro, M.D., Judith A. Hannan, Debra W. Jackson, Bruce L. Evatt, M.D., and Timothy J. Dondero, M.D.; and Laboratory Working Group: Gerald Schochetman, Ph.D. (chair), Charles A. Schable, M.S., J. Steve McDougal, M.D., J. Richard George, Ph.D., Thomas J. Spira, M.D., and Thomas M. Folks, Ph.D.

Address reprint requests to Dr. Smith at the Division of HIV/AIDS, Mailstop E-45, National Center for Infectious Diseases, Centers for Disease Control and Prevention, Atlanta, GA 30333.

Full Text of this Article

Related Letters:

CD4+ T-Lymphocytopenia without HIV Infection
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.
Extract | Full Text  
N Engl J Med 1993; 328:1847-1850, Jun 24, 1993. Correspondence

Idiopathic CD4+ T-Lymphocytopenia
DeHovitz J. A., Feldman J., Landesman S.
Extract | Full Text  
N Engl J Med 1993; 329:1045-1046, Sep 30, 1993. Correspondence

This article has been cited by other articles:

• Eneh, K. K., Zahir, M., Woldaregay, B., Hammoudeh, F., Narendra, N., Jeannot, M., Greenbery, G., Enriquez, D., Schmidt, F. (2009). DISSEMINATED MYCOBACTERIUM AVIUM INFECTION IN AN HIV-NEGATIVE PATIENT WITH NO EVIDENCE OF IMMUNOSUPPRESSION. Chest Meeting 136: 58S-58S [Abstract]  
• Thoden, J., Venhoff, N., Daskalakis, M., Schmitt-Graeff, A., Drager, R., Schlesier, M., Warnatz, K., Peter, H.-H. (2009). Disseminated tuberculosis in a patient with idiopathic CD4+ lymphocytopenia. Rheumatology (Oxford) 48: 1329-1330 [Full Text]  
• 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]  
• Baylor, P., Larson, R. (2009). Mycobacterium avium Septicemia With ARDS in a Patient With Diabetes Mellitus and No Other Known Immune-compromising Condition. J Intensive Care Med 24: 140-143 [Abstract]  
• 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]  
• Voisset, C., Weiss, R. A., Griffiths, D. J. (2008). Human RNA "Rumor" Viruses: the Search for Novel Human Retroviruses in Chronic Disease. Microbiol. Mol. Biol. Rev. 72: 157-196 [Abstract] [Full Text]  
• Carneiro-Sampaio, M., Coutinho, A. (2007). Immunity to Microbes: Lessons from Primary Immunodeficiencies. Infect. Immun. 75: 1545-1555 [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]  
• Daikos, G. L., Syriopoulou, V., Aperis, G., Toubanakis, C., Petrikkos, G., Demonakos, M. (2005). Disseminated Aspergillosis Mimicking Hepatic Veno-Occlusive Disease. ANN INTERN MED 143: 315-316 [Full Text]  
• Bharadwaj, J. S., Goulet, C. J., Tonnerre, C. (2005). The Unturned Stone. NEJM 352: 2140-2141 [Full Text]  
• Schattner, A., Friedman, J., Bentwich, Z. (2004). Opportunistic infection due to unexplained CD4+ lymphocytopenia and associated Sjogren's syndrome. Rheumatology (Oxford) 43: 111-112 [Full Text]  
• Cheung, M. C., Rachlis, A. R., Shumak, S. L. (2003). A cryptic cause of cryptococcal meningitis. CMAJ 168: 451-452 [Abstract] [Full Text]  
• Fry, T. J., Mackall, C. L. (2002). Interleukin-7: from bench to clinic. Blood 99: 3892-3904 [Full Text]  
• Kilic, S S., Bostan, O, Cil, E (2002). Takayasu arteritis. Ann Rheum Dis 61: 92-93 [Full Text]  
• Fry, T. J., Connick, E., Falloon, J., Lederman, M. M., Liewehr, D. J., Spritzler, J., Steinberg, S. M., Wood, L. V., Yarchoan, R., Zuckerman, J., Landay, A., Mackall, C. L. (2001). A potential role for interleukin-7 in T-cell homeostasis. Blood 97: 2983-2990 [Abstract] [Full Text]  
• Matsuyama, W., Mizoguchi, A., Iwami, F., Koreeda, Y., Wakimoto, J., Kanazawa, H., Mori, S., Kawabata, M., Fukunaga, H., Osame, M. (2000). Clinical investigation of pulmonary Mycobacterium avium complex infection in human T lymphotrophic virus type I carriers. Thorax 55: 388-392 [Abstract] [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]  
• Iwase, T., Ojika, K., Katada, E., Mitake, S., Nakazawa, H., Matsukawa, N., Otsuka, Y., Tsugu, Y., Kanai, H., Nakajima, K. (1998). An unusual course of progressive multifocal leukoencephalopathy in a patient with idiopathic CD4+ T lymphocytopenia. J. Neurol. Neurosurg. Psychiatry 64: 788-791 [Abstract] [Full Text]  
• Myskowski, P. L. (1997). Molluscum Contagiosum: New Insights, New Directions. Arch Dermatol 133: 1039-1041 [Abstract]  
• Couderc, L.-J., Caubarrere, I., Wiley, E. L., Nightingale, S. D., Mark, E. J. (1996). Tuberculous Bronchiolitis. NEJM 334: 1748-1749 [Full Text]  
• Sleasman, J.W. (1996). The Association Between Immunodeficiency and the Development of Autoimmune Disease. ADR 10: 57-61 [Abstract]  
• Rezza, G, Pezzotti, P, Aiuti, F (1995). Acquired immunodeficiency without HIV infection: epidemiology and clinical outcome in Italy. BMJ 311: 785-786 [Full Text]  
• Griffiths, T. W., Stevens, S. R., Cooper, K. D. (1994). Acute Erythroderma as an Exclusion Criterion for Idiopathic CD4+ T Lymphocytopenia. Arch Dermatol 130: 1530-1533 [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]  
• Ho, D. D., Cao, Y., Zhu, T., Farthing, C., Wang, N., Gu, G., Schooley, R. T., Daar, E. S. (1993). Idiopathic CD4+ T-Lymphocytopenia -- Immunodeficiency without Evidence of HIV Infection. NEJM 328: 380-385 [Abstract] [Full Text]  
• Spira, T. J., Jones, B. M., Nicholson, J., Lal, R. B., Rowe, T., Mawle, A. C., Lauter, C. B., Shulman, J. A., Monson, R. A. (1993). Idiopathic CD4+ T-Lymphocytopenia -- An Analysis of Five Patients with Unexplained Opportunistic Infections. NEJM 328: 386-392 [Abstract] [Full Text]  
• Duncan, R. A., von Reyn, C. F., Alliegro, G. M., Toossi, Z., Sugar, A. M., Levitz, S. M. (1993). Idiopathic CD4+ T-Lymphocytopenia -- Four Patients with Opportunistic Infections and No Evidence of HIV Infection. NEJM 328: 393-398 [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]  
• Vermund, S. H., Hoover, D. R., Chen, K. (1993). CD4+ Counts in Seronegative Homosexual Men. NEJM 328: 442-442 [Full Text]