Changes in von Willebrand Factor Multimer Distribution in Cirrhotic Patients with Acute Decompensation and Subsequent Stabilization

Background and aims: Acute decompensation (AD) of cirrhosis is a critical clinical event with heterogeneous outcomes, ranging from stable decompensated (SD) cirrhosis to acute-on-chronic liver failure (ACLF). Disturbances in the primary and secondary hemostasis have been implicated in the progression of cirrhosis. This study aimed to evaluate changes in primary hemostasis, i.e., VWF parameters, multimer distribution and ADAMTS13 activity in patients who could clinically improve after AD. Methods: Thirty-five cirrhotic patients hospitalized with AD were prospectively assessed at baseline and at 3-month follow-up when they reached an SD state. Clinical and laboratory characteristics, including the MELD score, were evaluated at both time points. VWF level and its different activities were measured using automated immunoassays, ADAMTS13 activity by chemiluminescent assay, and VWF multimer distribution using electrophoresis and densitometry. Results: At AD, at least one organ failure occurred in 13 patients, with 4 patients identified as ACLF grade 1. The MELD score was 22 (IQR 18–25) at AD, while at 3 months (SD) it was 17 (12–20) (p < 0.001). VWF parameters similarly decreased [VWF antigen 530% vs. 409% (p = 0.009), VWF activity 332% vs. 301% (p = 0.038), VWF collagen-binding 399% vs. 308% (p = 0.017)], while ADAMTS13 activity increased from 55% to 84% (p < 0.001). Ultra-large VWF multimers (ULMWM) were detected in six patients during AD but were absent in all SD samples, while ADAMTS13 activity normalized. Conclusions: Our findings demonstrate that the VWF–ADAMTS13 axis in cirrhosis is a dynamically changing marker, and the temporary appearance of ULMWM during AD exposes thrombogenic potential in cirrhosis.