Figure 3.
Exocytosis is defective in cytotoxic lymphocytes of FHL3-5, CHS, and GS2 patients. (A-C) Percentages of CD8+CD57+ T cells (A) or CD3–CD56dim NK cells (B-C) expressing surface CD107a after incubation with P815 cells with anti-CD3 antibody (A), P815 cells with anti-CD16 antibody (B), or K562 cells (C), shown for 5 different groups, as indicated. Values represent the difference in CD107a levels of stimulated against control unstimulated cells. Boxes display quartiles and whiskers display 2.5th and 97.5th percentiles, as well as outliers. Student t test comparing EXO against the other groups; ∗∗∗∗P < .0001. (D-E) Exocytosis data of specific patient categories from the “impaired exocytosis” and “other IEI patients” groups are shown individually. Each individual patient value is represented by a symbol, with violin plots indicating quartiles. Colored markings depict normal ranges from 198 healthy adults, with boxes displaying quartiles and whiskers displaying 2.5th and 97.5th percentiles. (E) Student t test comparing patients with 198 heathy adult controls; n.s., not significant; ∗P < .05; ∗∗P < .01; ∗∗∗∗P < .0001. CHS, Chediak-Higashi syndrome; FHL3, familial hemophagocytic lymphohistioccytosis type 3; XLP1, X-linked lymphoproliferative disease type 2.