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. 2022 May 11;6(6):e718. doi: 10.1097/HS9.0000000000000718

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Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the European Hematology Association.

This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.

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Figure 3. — Pathophysiology of reduced VWF levels are associated with VWF and FVIII levels and BS in type 1 VWD patients with a variant. (A, B) Patients with reduced synthesis/secretion or increased clearance have lower VWF:Ag and VWF:Ab than patients with undetermined pathophysiological defects of VWF. (C) Patients with increased clearance and both reduced synthesis/secretion and increased clearance of VWF also have lower FVIII:C compared to patients with undetermined pathophysiology. (D) BS is higher in patients with increased clearance of VWF compared with those with undetermined pathophysiological defects of VWF. Data are presented as mean and 95% CI and ¹reduced secretion of VWF. *P < 0.05, **P < 0.001. ns compared with patient with undetermined pathophysiological defects of VWF. Linear regression is used to compare each group with patients with undetermined pathophysiological defects of VWF. Regression analysis are adjusted for age, sex and blood group. Analysis with BS as outcome are not adjusted for blood group. BS = bleeding score; CI = confidence intervals; ns = not significant; Ref = reference; VWD = von Willebrand disease.