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.