Figure 2.

Flow-cytometric analysis. Cells in whole blood were stained for surface antigens, fixed, and permeabilized and then stained with PE-labeled WASP (shaded curves) or isotype control (open curves) mAbs. (A) Shown are total lymphocytes, T lymphocytes (CD3⁺), B lymphocytes (CD19⁺), and NK cells (CD3^-CD56⁺) of a genetically normal person (N), the revertant patient (P1), and his brother (P2). (B) Dot plot of patient (P1) NK cells stained with CD3 and CD56 and histograms showing WASP staining of CD56^bright and CD56^dim subpopulations. (C) Analysis of T lymphocytes, B lymphocytes, NK cells, and NK subsets of an (age-matched) genetically normal female and a female WAS carrier (large deletion of WASP gene). Note the presence among cells of the female carrier of approximately 88% WASP⁺ CD56^dim cells but only 78% WASP⁺ CD56^bright cells. Similar findings were obtained for a second carrier female (intron 6 + 5 g→a), 90% WASP⁺CD56^dim NK cells, 78% WASP⁺ CD56^bright cells.