Figure 2.

Functional response of the Wiskott-Aldrich syndrome and healthy platelets. (A-G) Whole blood platelets were stimulated (designated by A) or not (designated N/A) with thrombin receptor agonist peptide-6 plus collagen-related peptide and analyzed by flow cytometry. Parameters shown for healthy children (n=21, age 0-13 years, median 5.0) and Wiskott-Aldrich patients (17 treated with romiplostim, 11 non-treated) are: platelet size, determined from the forward scatter measured by mean fluorescence intensity (MFI) (A); CD42b level, MFI (B); CD61 level, MFI (C); PAC1-positive platelets, % (D); CD62p-positive platelets, % (E); dense granule release determined by mepacrine level, MFI (F); and phosphatidylserine-positive platelet fraction, % (G). P: Mann–Whitney U-test, P*:Wilcoxon signed-rank test. FSC: forward scatter, WAS: Wiskott-Aldrich syndrome; PS⁺: phosphatidylserine-positive.