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. 2023 Dec 27;143(14):1414–1424. doi: 10.1182/blood.2023022457

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© 2024 American Society of Hematology. Published by Elsevier Inc. Licensed under Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0), permitting only noncommercial, nonderivative use with attribution. All other rights reserved.

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Figure 2. — Pathophysiological mechanisms in patients with low VWF compared with type 1 VWD subgroups. FVIII:C/VWF:Ag and VWFpp/VWF:Ag ratios were assessed in patients with low VWF and type 1 VWD to assess underlying pathophysiology. (A) Significantly increased plasma FVIII:C/VWF:Ag ratios suggesting marked reductions in VWF synthesis/secretion were observed in the WiN persistent <30 IU/dL subgroup. In contrast, FVIII:C/VWF:Ag ratios were the same in LoVIC and WiN-normalized patients. (B) Similarly, significantly increased plasma VWFpp/VWF:Ag ratios suggesting markedly enhanced VWF clearance were also observed in the WiN persistent <30 IU/dL subgroup. Again, VWFpp/VWF:Ag ratios were not significantly different between LoVIC and WiN-normalized patients. Ns, not significant.