Von Willebrand Factor Levels in Patients With Liver Cirrhosis and Portal Hypertension

Patients with cirrhosis have an endotoxemic medium due to intestinal flora changes, decreased hepatic clearance of endotoxins and transition of bacteria to mesenteric lymph nodes from intestinal barrier .Serum Von Willebrand factor (vWF) increases in a linear ratio with the intensity of endotoxemia.

Portal hypertension (PH) is a hallmark of advanced chronic liver disease (ACLD) that significantly influences the onset of liver-related complications and mortality. It can be quantified using the minimally invasive measurement of the hepatic venous pressure gradient (HVPG). Experimental research has demonstrated that ongoing dysfunction of liver sinusoidal endothelial cells (LSEC) triggers the activation of hepatic stellate cells (HSC) and leads to intrahepatic vasoconstriction, thereby contributing to PH.

Von Willebrand factor (VWF) is released by activated endothelial cells and plays a crucial role in hemostasis by facilitating the adhesion of platelets to subendothelial collagen and other platelet-adhesive proteins exposed during vascular injury. Early studies revealed that VWF levels are elevated in patients with ACLD, linking this finding to endothelial dysfunction likely caused by endotoxemia , resulting from pathological bacterial translocation. Notably, VWF antigen (VWF-Ag) has shown diagnostic potential for clinically significant PH (CSPH; defined by an HVPG ≥ 10 mmHg) and prognostic value for disease progression, even after the underlying cause has been treated.

Elevated VWF levels may help balance the fragile hemostatic system in patients with ACLD by compensating for platelet deficiencies in number and possibly function.

VWF is more than just a hemostatic protein; it serves as a marker of endothelial dysfunction in patients with cirrhosis.VWF levels can independently predict decompensation and mortality in cirrhosis patients, regardless of the stage of liver disease and severity of portal hypertension. The VWF-to-thrombocyte ratio (VITRO) score enhances the diagnostic capability of VWF alone in noninvasively detecting CSPH.In addition to predicting the risk of hepatocellular carcinoma (HCC) in cirrhosis patients, VWF levels also forecast the risk of complications following HCC resection and response to systemic therapies.