Page 257 - Haematologica April 2020

Programmed necrosis of platelets in WAS
AB
CD
EF
G
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 throm- bin receptor agonist peptide-6 plus collagen-related peptide and analyzed by flow cytome- try. 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-pos- itive platelet fraction, % (G). P: Mann–Whitney U-test, P*:Wilcoxon signed-rank test. FSC: for- ward scatter, WAS: Wiskott-Aldrich syndrome; PS+: phosphatidylserine-positive.
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