WHIM patients harbor quantitative defects in pDCs. (A) Blood DC subsets were analyzed in WHIM patients and healthy donors. Representative pseudo-color dot plots obtained for the same volume of blood from 1 healthy control (control) and 1 WHIM patient (WS) carrying the heterozygous CXCR4^+/1013 mutation are shown. cDC2 was defined as CD11c⁺HLA-DR⁺CD1c⁺ cells, cDC1 as CD11c⁺HLA-DR⁺CD141⁺ cells, and pDCs as CD11c⁻HLA-DR⁺CD303⁺ cells. Percentages among parent cells are indicated. (B-C) The frequency of DC subsets among peripheral blood mononuclear cells (B) and their concentrations (C) were measured for healthy controls (control) and WHIM patients (WS). Each point represents an individual and lines indicate the mean ± standard error of the mean (SEM). n = 9 Controls and n = 4 WS (B); n = 7 controls and n = 4 WS (C). Statistical analysis was performed using the 2-tailed, unpaired Mann-Whitney test. *P < .05, **P < .01.