Ex vivo platelet aggregation and blood plasma
coagulation
4/7 patients (Patients 2, 3, 6 and 7) demonstrated almost normal blood
plasma coagulation. P1 demonstrated significant hypercoagulation, and
Patients 4,5 and 6 demonstrated mild hypocoagulation (Fig. S1). These
data are consistent with the presence of KMP and DIC-like phenotype in
P1 and the previously reported reduction of D-dimer levels upon
sirolimus administration 33. Altogether we conclude
that KHE does not significantly affect blood coagulation.
Ex vivo platelet aggregation in collagen covered parallel-plate
flow chambers was performed as described previously27. In accordance with previously published results,
thrombi in the samples from the thrombocytopenic patient (P1) were
significantly smaller than those of healthy donors (Fig. 2A). However,
thrombi formed in the samples of patients with KHE
(non-thrombocytopenic) were also noticeably smaller than those from
healthy donors’ samples (Fig. 2B-D).