The increase of non-MHC-restricted cytotoxic cells (gamma/delta-TCR-bearing T cells or NK cells) and the abnormal differentiation of B cells in Wiskott-Aldrich syndrome.
The objective of this study was to analyze the configuration of the lymphocytes in Wiskott-Aldrich syndrome (WAS) by studying the surface antigens from nine cases using dual-color immunofluorescence analysis. All the patients showed the increase of non-MHC-restricted cytotoxic cells, namely CD3+ WT31- delta TCS1+ (gamma/delta-T cell receptor (TCR)-bearing cells) and/or CD16+ natural killer cells. The gamma/delta-TCR+ cells of WAS, however, were unique since they did not express CD5, which is present on ordinary gamma/delta-TCR+ cells. A reduced number of CD4+ cells and an increased percentage of CD11b+ Leu7+ cells within a CD8+ subset were observed in all cases. With regard to B cell subpopulations, most cases showed reduced Fc epsilon R2-bearing B cells, despite an elevated serum IgE.
Morio
T
T
Department of Pediatrics, Faculty of Medicine, Tokyo Medical and Dental University, Japan.
Takase
K
K
Okawa
H
H
Oguchi
M
M
Kanbara
M
M
Hiruma
F
F
Yoshino
K
K
Kaneko
T
T
Asamura
S
S
Inoue
T
T
D000293:Adolescent; D000328:Adult; D000954:Antigens, Surface / Q000032:analysis; D001402:B-Lymphocytes / Q000145:classification*; D002648:Child; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D007694:Killer Cells, Natural / Q000145:classification*; D008297:Male; D011948:Receptors, Antigen, T-Cell / Q000032:analysis; D016692:Receptors, Antigen, T-Cell, gamma-delta; D013601:T-Lymphocytes / Q000145:classification*; D014923:Wiskott-Aldrich Syndrome / Q000276:immunology*
D000954:Antigens, Surface; D011948:Receptors, Antigen, T-Cell; D016692:Receptors, Antigen, T-Cell, gamma-delta