Blood ANCs of individuals with ADAN. (A) ANCs of 66 individuals with ADAN, grouped according to the NP strata as severe (0-0.49 × 109/L), moderate (0.5-0.99 × 109/L), mild (1.0-1.49 × 109/L), and borderline (1.50-1.60 × 109/L) NP. The median ANCs are shown in the left panel, and the lowest-recorded ANC values during the observation time are shown in the right panel. The numbers above the bars denote the number of subjects in that stratum. (B) Increase in the ANC at the time of simultaneous recorded low, moderate, or marked CRP increases. The graph is based on available data from 13 individuals (1-5 independent samples per subject) for which CRP levels and ANCs were analyzed simultaneously when persons with ADAN sought medical care for minor infectious/inflammatory conditions (eg, upper respiratory tract infections). ANC and CRP levels did not correlate statistically when removing an outlier. (C) All recorded ANCs for 2 individuals with ADAN followed up for 5 and 8 years, respectively. Light red bars depict ANC when the CRP was raised (according to the inserted explanation). Red bars represent ANC obtained at normal CRP values. ANC levels for subject A remained stable over the individual follow-up period (however, short periods with concomitantly raised ANC and CRP values were noted). This patient remained in excellent health, displaying a normal pro-LL-37 plasma value, a normal BM sample, and whole-genome sequencing showing no other NP-related mutations/variants.
