TY - JOUR
T1 - Behcet's-like syndrome associated with idiopathic CD4+ T- lymphocytopenia, opportunistic infections, and a large population of TCRαβ+ CD4- CD8- T cells
AU - Venzor, Joe
AU - Hua, Qiang
AU - Bressler, Robert B.
AU - Miranda, Charles H.
AU - Huston, David P.
PY - 1997
Y1 - 1997
N2 - Herein we report a patient with Behcet's like syndrome, idiopathic CD4+ T-lymphocytopenia, opportunistic infections, and a large polyclonal population of TCRαβ+ 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% TCRαβ+, of which 74 ± 5% were CD4- CD8- . No clonal populations were detected by southern analysis for T-cell receptor Vβ 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 TCRαβ+ 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 Behcet's-like syndrome with lethal consequences.
AB - Herein we report a patient with Behcet's like syndrome, idiopathic CD4+ T-lymphocytopenia, opportunistic infections, and a large polyclonal population of TCRαβ+ 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% TCRαβ+, of which 74 ± 5% were CD4- CD8- . No clonal populations were detected by southern analysis for T-cell receptor Vβ 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 TCRαβ+ 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 Behcet's-like syndrome with lethal consequences.
KW - Behcet's syndrome
KW - CD4 CD8 T cells
KW - Idiopathic CD4 T-lymphocytopenia
KW - Systemic nocardiosis
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U2 - 10.1016/s0002-9629(15)40100-4
DO - 10.1016/s0002-9629(15)40100-4
M3 - Article
C2 - 9099154
AN - SCOPUS:0030611715
SN - 0002-9629
VL - 313
SP - 236
EP - 238
JO - American Journal of the Medical Sciences
JF - American Journal of the Medical Sciences
IS - 4
ER -