Von Willebrand Factor and Prognosis of Patients with Amyloid Light-Chain Amyloidosis

Amyloid fibrils are toxic to the endothelium, and their deposition in the heart, kidneys, and liver severely damages these organs. Von Willebrand factor is a biomarker for endothelial cell injury and might indicate the extent and severity of amyloid light-chain (AL) amyloidosis.

To determine whether high levels of von Willebrand antigen (VWF:Ag) are associated with a poor prognosis, investigators studied 111 newly diagnosed patients with AL amyloidosis prior to treatment. Most had stage II or III disease, and 19% had stage IIIB and N-terminal pro-brain natriuretic peptide (NTproBNP) >8500 pg/mL.

The median level of VWF:Ag was significantly higher in patients than in healthy controls (203 U/dL vs. 84 U/dL; P<0.001). VWF:Ag levels correlated wi…