Idiopathic CD4+ T-Lymphocytopenia -- An Analysis of Five Patients with Unexplained Opportunistic Infections

Abstract

Idiopathic CD4+ T-lymphocytopenia differs from HIV infection in its immunologic characteristics and in its apparent lack of progression over time. Nothing about the immunologic or viral-culture studies performed in these patients or about their family members or blood donors suggests that a transmissible agent causes this condition.

Apoptotic depletion of CD4+ T cells in idiopathic CD4+ T lymphocytopenia.

“…Marked CD4 ϩ T lymphopenia in the absence of HIV infection has been documented by our group (1) and others (2,3). This syndrome is termed idiopathic CD4 ϩ T lymphocytopenia (ICL) 1 by the U.S. Centers for Disease Control and Prevention (2), and severe unexplained HIV seronegative immune suppression (SUHIS) by the World Health Organization (4).…”

“…This syndrome is termed idiopathic CD4 ϩ T lymphocytopenia (ICL) 1 by the U.S. Centers for Disease Control and Prevention (2), and severe unexplained HIV seronegative immune suppression (SUHIS) by the World Health Organization (4). It is defined by an absolute CD4 count Ͻ 300/mm 3 or Ͻ 14%, either alone (ICL) or accompanied by clinical signs and symptoms of cellular immune deficiency (SUHIS).…”

Idiopathic CD4+ T-Lymphocytopenia with Bowen's Disease.

“…In 1992, the Centers for Disease Control and Prevention (CDC) termed this condition idiopathic CD4+ T-lymphocytopenia (ICL) and provisionally defined it for adults as follows (1): 1) CD4+ T-lymphocyte depletion (absolute CD4+T-cell level <300 cells/pl or <20%of total lymphocytes on more than one determination), 2) no serologic evidence of HIV infection, and 3) no defined immunodeficiency or therapy associated with T-cell depletion. About 70 cases of ICL have been reported in the USA, Europe and Australia (2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12). In contrast, there have been a few reports in Japan (13,14).…”

Safety and efficacy of treatment using interleukin-2 in a patient with idiopathic CD4+ lymphopenia andMycobacterium avium-intracellulare

“…+ lymphopenia (ICL) is a rare immune defect described first in a series of early 1990s case reports and characterized further by analysis of HIV-seronegative patients in the national AIDS reporting system and blood transfusion safety studies [1][2][3][4][5][6][7]. The Center for Disease Control established a working definition of ICL including the following criteria: (1) CD4 + T lymphopenia (CD4 + cell count < 300 cells/mm 3 or CD4 + cell count < 20% of T cells) on more than one occasion; (2) lack of HIV infection; and (3) no immune deficiency, infection or therapy associated with T cell lymphopenia [1].…”

“…The Center for Disease Control established a working definition of ICL including the following criteria: (1) CD4 + T lymphopenia (CD4 + cell count < 300 cells/mm 3 or CD4 + cell count < 20% of T cells) on more than one occasion; (2) lack of HIV infection; and (3) no immune deficiency, infection or therapy associated with T cell lymphopenia [1]. In most reported cases, ICL patients present with opportunistic infections including: extrapulmonary and pulmonary Mycobacterium avium complex, Pneumocystis jiroveci pneumonia, cryptococcal meningitis, oral candidiasis, disseminated cutaneous Herpes zoster, cerebral toxoplasmosis and numerous other opportunistic infections [1][2][3][4][5][6]8]. However, some ICL patients do not have opportunistic infections and are relatively healthy [8,9].…”