Subsets of mDCs and pDCs in XLA patients. (A) Peripheral blood DC subsets in XLA patients and healthy controls. Myeloid DCs (mDCs) were identified as lineage-negative/HLA-DR⁺/CD11c⁺ cells and plasmacytoid DCs (pDCs) as lineage-negative/HLA-DR⁺/CD123⁺ cells. Absolute numbers of circulating DC subsets in peripheral blood are shown. Points correspond to individual patients; horizontal bars represent the mean value for the group. (B) Expression of TLRs 1 to 9 on immature DCs from XLA patients and healthy blood donors analyzed by RT-PCR. Both XLA-DC and control DCs expressed TLRs 1 to 6 and 8, a profile characteristic of myeloid DCs. As expected, we did not detect TLR-7 and TLR-9, molecules typically expressed on plasmacytoid DCs. Representative results of 1 of 5 independent experiments are shown. (C) Characteristics of DCs in XLA patients. (i) CD86 expression (thick line) versus isotype control (thin line) is shown. Representative results of 1 of 10 experiments are shown. (ii) Summary of CD86 expression on XLA-DCs, control DCs, and control DCs plus LFM-A13 after activation with TLR agonists. Average of mean fluorescent intensity values plus SDs for 5 different XLA patients and healthy controls is shown. (D) Endocytic activity of XLA-DCs and DCs from healthy donors after stimulation with TLR agonists.