Skewed surface KIR receptor expression in patients with NK-LDGL. Dot plot flow cytometry results for enriched normal NK cells (left column), PBMCs from NK-LDGL3 (middle column), and PBMCs from NK-LDGL6 (right column) after staining with (A) isotype control-CyChrome (y-axis, control for normal NK and NK-LDGL3) and isotype control-FITC (y-axis, control for NK-LDGL6) and/or isotype control-PE (x-axis, control). Two-color analysis is shown for either anti-CD56-CyChrome (NK-LDGL3 and normal NK) or anti-CD16-FITC (NK-LDGL6) with (B) anti-CD158a-PE (KIR2DS1 and KIR2DL1), (C) anti-CD158b-PE (KIR2DL2, KIR2DL3, and KIR2DS2), (D) anti-NKB1-PE (KIR3DL1), (E) anti-KARp50-PE (KIR2DS4), and (F) anti-CD94-PE. The percentage of cells positive in each quadrant is indicated in the top right-hand corner of each dot plot. Adding the numbers in the top left and top right quadrants represents the total number of NK cells (∼48% for NK-LDGL3 and ∼83% for NK-LDGL6). The number in the top right represents the number of NK cells coexpressing the NK receptor. R1 indicates region 1; Inh, inhibitory KIR; and Ac, activating KIR.