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Update: CD4+ T-Lymphocytopenia in Persons Without Evident HIV Infection -- United States

On July 31, 1992, CDC reported five cases of CD4+ T-lymphocytopenia in persons without evident human immunodeficiency virus (HIV) infection in the United States (1). As of August 5, 1992, CDC has received reports of nine additional persons with similar clinical presentations. All persons who have been reported to CDC meet the three criteria for CD4+ T-lymphocytopenia without evident HIV infection. * Another 21 persons suspected to have this condition have been described (1), 10 of whom reside in the United States. This report summarizes the 14 cases reported to CDC and provides information on the national surveillance system established to determine the prevalence and distribution of this condition. **

The 14 persons reported to CDC resided in 10 states, and their CD4+ T-lymphocytopenia was first documented during 1985-1992. These persons ranged in age from 31 to 70 years (median: 48 years); eight (57%) were male. Twelve persons (86%) were white, one (7%) black, and one (7%) Asian.

Information about risk factors for HIV infection was available for 13 persons, of whom four (31%) had established risk factors: three persons had received blood transfusions, and one person reported male homosexual contact. Acquired immunodeficiency syndrome (AIDS)-defining illnesses were diagnosed in eight (57%) of the 14 persons (2); six had other illnesses. One person died from an AIDS-defining illness; the other 13 are alive.

The lowest recorded CD4+ T-cell levels were 17-200 cells/uL (median: 85 cells/uL). In addition to testing for antibody to HIV, supplemental tests for HIV infection were performed for seven of the 14 persons and were negative. These supplemental tests included polymerase chain reaction for HIV DNA sequences (five persons), coculture of peripheral blood monocytes (three), and HIV p24 antigen assay (six).

The 10 U.S. cases previously described (3-5) are under investigation. A summary of information obtained to date indicates that eight of the 10 persons were male. Risk factors for HIV infection included male homosexual contact (six) and receipt of blood transfusions (one); three had no reported risk factors. Three persons had AIDS-defining illnesses, three had other illnesses, and four were asymptomatic. Of nine persons for whom vital status was known, two died from AIDS-defining illnesses. All 10 persons had at least one supplemental test for HIV infection; all of these tests were negative. All six persons with documented CD4+ T-cell levels had less than 300 cells/uL.

Reported by: H Kessler, MD, Rush Presbyterian-St. Luke's Medical Center, Chicago. R Duncan, MD, Boston City Hospital, Boston. T Blok, MD, Parkside Internal Medicine, Kalamazoo, Michigan. C von Reyn, MD, Infectious Disease Section, Dept of Medicine, Dartmouth-Hitchcock Medical Center, Lebanon, New Hampshire. C Farthing, MD, New York Univ Medical Center, New York City. B Jones, DVM, Div of Epidemiology, Bur of HIV/AIDS, Pennsylvania Dept of Health. Div of HIV/AIDS, National Center for Infectious Diseases, CDC.

Editorial Note

Editorial Note: HIV-negative persons with apparent CD4+ T-lymphocytopenia are under epidemiologic and laboratory investigation by CDC and the National Institutes of Health. The cause of this condition remains unknown; these cases may represent a heterogeneous group of disorders.

In collaboration with state and local health departments, CDC has developed a standardized national surveillance system for collecting and reporting information on HIV seronegative persons with CD4+ T-lymphocyte depletion. Health-care providers are requested to report such cases to CDC through the AIDS surveillance section of their local or state health departments. Additional information on case reporting is available from CDC (telephone (404) 639-2981). Investigators in charge of Public Health Service-sponsored clinical trials and epidemiologic cohort studies, members of the Infectious Disease Society of America, the National Hemophilia Foundation, laboratories participating in CDC's Model Performance Evaluation Program, and physicians/institutions who report persons with HIV infection/AIDS are being contacted directly to facilitate reporting of cases to this surveillance system.

A scientific meeting will be held on August 14, 1992, at CDC to review the findings from these investigations. Additional information about the meeting and registration is available from PACE Enterprises; telephone (404) 633-8610.


  1. CDC. Unexplained CD4+ T-lymphocyte depletion in persons without evident HIV infection -- United States. MMWR 1992;41:541-5.

  2. CDC. Revision of the CDC surveillance case definition for acquired immunodeficiency syndrome. MMWR 1987;36(suppl 1).

  3. Daar ES, Moudgil T, Ho DD. Persistently low T-helper (CD4+) lymphocyte counts in HIV-negative asymptomatic men. Western Society of Clinical Investigation meeting, February 1990.

  4. Laurence J, Siegal FP, Schattner E, Gelman IH, Morse S. Acquired immunodeficiency without evidence of infection with human immunodeficiency virus types 1 and 2. Lancet 1992;340:273-4.

  5. Gupta S, Ribak CE, Gollapudi S, Kim CH, Salahuddin SZ. Detection of a human intracisternal retroviral particle associated with CD4+ T-cell deficiency. Proc Natl Acad Sci USA (in press).

    • Single copies of this report will be available free until August

    7, 1993, from the CDC National AIDS Clearinghouse, P.O. Box 6003, Rockville, MD 20849-6003.

    • Low CD4+ T-cell levels (documented absolute CD4+ T-cell level less than 300 cells/uL OR less than 20% on more than one determination); negative laboratory evidence of HIV infection (includes HIV serology and, if performed, HIV p24 antigen, polymerase chain reaction, and viral culture); and no defined immunodeficiency or therapy associated with depressed CD4+ T-cell levels. ** Single copies of this report will be available free until August 7, 1993, from the CDC National AIDS Clearinghouse, P.O. Box 6003, Rockville, MD 20849-6003.

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