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[Idiopathic CD4+ lymphocytopenia: a case report]
M A Frassanito1, G Iodice, R Rizzi
1Dipartimento di Scienze Biomediche ed Oncologia Umana, Università degli Studi di Bari.
Annali Italiani Di Medicina Interna : Organo Ufficiale Della Societa Italiana Di Medicina Interna
|July 1, 1995
Summary
Idiopathic CD4+ lymphocytopenia (ICL) is a rare condition characterized by low CD4+ T cells without HIV infection. This case suggests increased lymphocyte apoptosis may underlie ICL, impacting immune function.
Area of Science:
- Immunology
- Virology
Background:
- Recurrent opportunistic infections can indicate severe immune deficiency.
- Human Immunodeficiency Virus (HIV) infection is a common cause of CD4+ T cell depletion.
Observation:
- A 47-year-old patient presented with recurrent opportunistic infections and a severe reduction in CD4+ T cells.
- Standard HIV testing (Elisa, Western blot, PCR) repeatedly yielded negative results.
- Laboratory findings included low CD4+ T cells without elevated CD8+ T cells or hypergammaglobulinemia.
Findings:
- The patient met the diagnostic criteria for idiopathic CD4+ lymphocytopenia (ICL): CD4+ T cells < 300/mm³ on two occasions and no HIV evidence.
- In vitro studies showed impaired lymphocyte proliferation in response to mitogens (concanavalin A, pokeweed mitogen).
- Increased Fas antigen expression and a higher percentage of apoptotic cells were observed in peripheral lymphocytes.
Implications:
- The findings suggest that increased lymphocyte apoptosis, potentially triggered by an in vivo signal, may be a mechanism underlying ICL.
- Understanding the pathogenesis of ICL is crucial for managing patients with unexplained immune deficiencies.
- This case highlights the importance of considering ICL in the differential diagnosis of cellular immunodeficiency when HIV is excluded.