Figure 2.

Pathophysiology of reduced VWF levels in patients with type 1 VWD with a single variant. Each dot represents the mean value for a variant. Triangles indicate variants with undetermined pathophysiological defects of VWF. Subscript number indicate number of patients with each variant. FVIII:C/VWF:Ag ratio ≥1.9 is defined as reduced synthesis/secretion of VWF and VWFpp/VWF:Ag ratio ≥2.2 is defined as an increased clearance of VWF. Of note, the number of patients with each variant in this figure may differ from the number of patients presented in Suppl. Table S1, because in this figure, we only present type 1 VWD patients with a single variant. VWD = von Willebrand disease.