Highly differentiated phenotype of the peripheral lymphoid compartment of patients with GATA2 mutation. (A) Example of B-cell profile of patient with GATA2 mutation compared with control according to published descriptions.^23,27  Populations: (1) transitional; (2) naïve mature; (3) mature activated; (4) resting memory; (5) plasmablast; and (6) CD38⁻CD21⁻ (autoimmune-associated). (B) Quantification of GATA2-mutated patients vs controls showing depletion of transitional B cells and naïve memory B cells, and accumulation of memory B cells and CD38⁻CD21⁻ B cells. (C) Example of NK-cell profile of patient with GATA2 mutation compared with control showing the distribution of CD56^bright NK cells, and NKG2A⁺ and KIR⁺ cells within the CD56^dim population. (D) Quantification of GATA2-mutated patients vs controls showing CD56^bright NK cells and the expression of differentiation-associated antigens within the CD56^dim population. Cytomegalovirus seropositivity is indicated by open symbols. (E) Example of CD3⁺ T-cell profile of a patient with GATA2 mutation compared with control showing CD4:CD8 profile and differentiation according to expression of CCR7 and CD45RA. (F) Quantification of antigen expression by CD8⁺ T cells of GATA2-mutated patients vs controls showing the acquisition of a terminally differentiated phenotype and increased expression of KIR on the CD56⁺ subset. (G) CD8⁺ CD161⁺ Va7.2⁺ MAIT cells are decreased in patients relative to controls. *P < .05; **P < .01; ***P < .001.