Figure 4.

Relative frequencies of B cells subsets in PIRD patients. The frequency of CD38⁺⁺CD24⁺⁺ cells (TBL, A), IgD⁺CD27^- cells (naïve B cells, B) CD19⁺CD21^lowCD38^+/- cells (CD21^low B cells, C), IgD^-IgM^-CD38^+/- cells (Sw-MBL, D) and CD38⁺⁺CD27⁺⁺ cells (PBC, E) in a cohort of HD (n = 5 for TBL, n = 11 for naïve B cells, n = 9 for CD21^low B cells, n = 13 for Sw-MBL and n = 6 for PBC) and 14 patients with dysregulatory syndrome PID were depicted. Horizontal lines represent the mean of each group. The distribution of the frequencies of CD38⁺⁺CD24⁺⁺ cells (TBL, F), IgD⁺CD27^- cells (naïve B cells, G) CD19⁺CD21^lowCD38^+/- cells (CD21^low B cells, H), IgD^-IgM^-CD38^+/- cells (Sw-MBL, I), and CD38⁺⁺CD27⁺⁺ cells (PBC, J) in each group of this cohort were also depicted. P1 (STAT1 GOF) and P11 (CVID) were under treatment (as detailed in Supplementary Table 1). Gray areas indicate the 10^th and 90^th percentiles of HD for each parameter. An unpaired non-parametric t-Student test (Mann-Whitney U test) was used in (A–E); a non-parametric test with Dunn’s post hoc test was used in (F–J). *p < 0.05; **p < 0.01; ***p < 0.001.