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).