Table 1.
Characterization and bleeding events of the studied patients.
Figure 1.
Thrombin generating capacity in plasma of the controls and cases.
Thrombin generation was measured in plasma triggered with 20 pM TF using the CAT method. Curve parameters are lag time (A) and peak height (B) of thrombin generation, and endogenous thrombin potential (ETP) (C). Normal ranges (plasmas from healthy subjects) are represented by dotted lines. Indicated are medians with interquartile ranges.
Figure 2.
vWf levels and activity in controls and cases.
Antigen levels (vWf:Ag, A) and ristocetin cofactor activity (vWf:RCoF, B) of vWf were determined in patients plasmas, and the ratio (C) was calculated. Normal ranges (plasmas from healthy subjects) are indicated by dotted lines. Data are shown as median levels with interquartile ranges.
Figure 3.
Platelet aggregation for controls and cases in response to a standard agonist panel.
Aggregation in PRP (250×109 plts/L) was determined in response to a streamlined panel of agonists: ADP (5 or 10 µM), collagen (1 or 4 µg/mL), SFLLRN (15 µM), epinephrine (10 µM), ristocetin (1.5 mg/mL) or arachidonic acid (1 mM). Representative aggregation traces are shown for controls (grey) and cases (black).
Figure 4.
Maximal aggregation extent for all controls and cases.
Quantification of the results depicted in figure 2. Maximal aggregation is shown (median with interquartile range). Normal ranges for platelets from healthy subjects are indicated by the dotted lines.
Figure 5.
Agonist-induced secretion and integrin activation of platelets from controls and cases.
PRP diluted in Hepes buffer was activated with 10 µM ADP, 50 ng/mL convulxin or 15 µM SFLLRN. Flow-cytometric detection of α-granule secretion using FITC-labeled P-selectin (A, D), dense-granule secretion with APC-labeled anti-CD63 (B, E), and platelet fibrinogen binding with FITC-labeled anti-fibrinogen mAb (C, F). Data are mean fluorescence intensity (MFI, left panels) and fractions of positive platelets (right panels). Medians with interquartile ranges.