PB1006 - Prognostic value of VWF and ADAMTS13 in progression of liver cirrhosis

Background: Studies have found that vWF antigen in patients with liver cirrhosis is higher than that in normal people, and increases with the severity of liver cirrhosis, which is associated with poor short-term prognosis. ADAMTS13 activity decreases with disease progression, especially in autoimmune cirrhosis.

Aims: To investigate the changes in von Willebrand factor(vWF) and ADAMTS13 in the progression of liver cirrhosis and to explore the mechanism of vWF function and vWF/ADAMTS13 changes in liver cirrhosis.

Methods: Thirty patients with cirrhosis in compensatory stage and decompensatory stage respectively were enrolled in the study, whose clinical data were collected. The vWF antigen(vWF:Ag), vWF collagen binding capacity(vWF:CB)，ADAMTS13 activity and the distribution of vWF multimers were studied. vWF antigen and activity were determined by ELISA. The multimers analysis was carried out using HYDRAGEL 5 von Willebrand Multimers kit on semi-automatic gel electrophoresis instrument HYDRASYS (SEBIA). ADAMTS13 activity was determined by fluorescein resonance energy transfer analysis, using the ACTIFLUOR™ ADAMTS13 Activity Assay (Biomedica)

Results: vWF:Ag and vWF:CB were higher in patients with decompensated cirrhosis than those in patients with compensated cirrhosis. ADAMTS13 activity decreased in decompensated patient. Compared with the compensated group, the proportion of low molecular weight multimers in vWF increases, while the proportion of high molecular weight multimers decreases in decompensated patients. Ultra large molecular weight multimers were discovered in some patients with decompensated cirrhosis.

Conclusion(s): Both plasma levels of vWF and binding function rise with disease progression in cirrhosis, but the two change asynchronously, indicating a relative decline in vWF function. The elevation of vWF:Ag/ADAMTS13 with disease cirrhosis disease progression and the increasing imbalance between the two suggest that there is a shift from hemostatic rebalance to hypercoagulability in the patient. Alterations in vWF with ADAMTS13 may be involved in the inflammation that accompanies cirrhosis.