Behçet's-like syndrome associated with idiopathic CD4+ T-lymphocytopenia, opportunistic infections, and a large population of TCR alpha beta+ CD4- CD8- T cells.

Abstract

Herein we report a patient with Behçet's like syndrome, idiopathic CD4+ T-lymphocytopenia, opportunistic infections, and a large polyclonal population of TCR alpha beta + CD4- CD8- T cells. Microfluorimetric analysis of peripheral blood mononuclear cells revealed CD4+ T-cell counts of 10 +/- 5/mm3. The CD3+ T cells were 99% TCR alpha beta +, of which 74 +/- 5% were CD4- CD8-. No clonal populations were detected by southern analysis for T-cell receptor V beta gene rearrangements. No evidence of human immunodeficiency virus infection was present, although nocardia, candida, pneumocystis, cytomegalovirus, and herpes infections were documented. The concomitant presence of opportunistic infections and a large population of TCR alpha beta + CD4- CD8- T cells suggests a pathogenic association and an intense immune response to microbial lipid or lipoglycan antigens presented in the context of CD1 molecules. This case demonstrates the potential for idiopathic CD4+ T-lymphocytopenia to occur in Behçet's-like syndrome with lethal consequences.

• Authors: J Venzor, Q Hua, R B Bressler, C H Miranda, D P Huston
• Journal: The American journal of the medical sciences (Am J Med Sci) Vol. 313 Issue 4 Pg. 236-8 (Apr 1997) ISSN: 0002-9629 [Print] United States
• PMID: 9099154 (Publication Type: Case Reports, Journal Article)

Chemical References

• Receptors, Antigen, T-Cell, alpha-beta

Topics

• Behcet Syndrome (complications, diagnosis, immunology)
• Candidiasis (complications, immunology)
• Cytomegalovirus Infections (complications, immunology)
• Fatal Outcome
• HIV Seronegativity
• Herpesviridae Infections (complications, immunology)
• Humans
• Male
• Middle Aged
• Nocardia Infections (complications, immunology)
• Nocardia asteroides
• Opportunistic Infections (complications, immunology)
• Pneumonia, Pneumocystis (complications, immunology)
• Receptors, Antigen, T-Cell, alpha-beta (metabolism)
• T-Lymphocyte Subsets (immunology)
• T-Lymphocytopenia, Idiopathic CD4-Positive (complications, immunology)