(a) Flow cytometry of circulating IgDlowCD27+ MZ B cells, IgDhighCD27− naive B cells and CD19+ total B cells from patients with neutrophil disorders (ND) and age-matched healthy donors (HD). SCN, severe congenital neutropenia; SBDS, Shwachman-Bodian-Diamond syndrome; WHIM, warts-hypogammaglobulinemia-infections-myelokatexis syndrome: CN, cyclic neutropenia; CGD, chronic granulomatous disease; LAD-1, leukocyte adhesion deficiency-1. ELANE, WASP, SBDSP1, CXCR4, CYBB and ITGB2 indicate genes encoding elastase, Wiskott-Aldrich syndrome protein, SBDS protein 1, CXCR4, p91-PHOX and CD18, respectively. (b) ELISA of IgM, IgG and IgA to LPS from Escherichia coli, LTA and PGN from Bacillus subtilis, or tetanus toxin (TT) from serum of patients and age-matched healthy donors as in a. OD, optical density. (c) Number of VH3–23 gene mutations per 100 base pairs (bp) in circulating MZ B cells from patients with SCN and an age-matched healthy donor. (d) Immunofluorescence of normal and SCN spleens stained for elastase (ELA, green), MR (red) and IgD (blue). Original magnification, ×10 (first and third panel from top) and ×63 (second and fourth panels from top). Error bars, median and percentile 25 and 75 (a, b) or s.e.m. (c); * P < 0.05 (Mann-Whitney U test). Data are from one of three experiments with similar results (d) or summarize multiple measurements (a–c).