Elevated Von Willebrand factor propeptide for the diagnosis of thrombotic microangiopathy and for predicting a poor outcome

Abstract

Thrombotic microangiopathy (TMA) is associated with vascular endothelial cell injury and is sometimes linked with poor outcome. Von Willebrand factor (VWF) propeptide (VWFpp) is considered to be a marker of vascular endothelial cell injury. The plasma levels of VWF, VWFpp, and thrombomodulin (TM) were evaluated for their use in the diagnosis of TMA in 75 patients with TMA. There were 30 TMA patients with marked decreases in ADAMTS13 (TMA/ADAMTS13) and 45 without the decrease (TMA/other). The plasma levels of TM, VWF, and VWFpp values were significantly high in patients with TMA, especially TMA/other group. The plasma levels of TM and VWFpp were significantly high in non-survivor with TMA. In the TMA/other group, the plasma levels of VWFpp were negatively correlated with ADAMTS13 activity. The plasma levels of TM correlated with the renal function, but the plasma levels of VWFpp did not. A ROC analysis indicated that VWFpp and TM were useful markers for the prediction of a poor outcome. These findings suggest that VWFpp is an useful marker for the diagnosis of TMA and for the prediction of poor outcome.

von Willebrand factor propeptide to antigen ratio in acquired thrombotic thrombocytopenic purpura

“…TTP patients in remission also had significantly higher VWF:Ag and VWFpp levels than controls, irrespective of the residual ADAMTS13 activity. The observation that patients with an ADAMTS13 activity of < 10% and those with an ADAMTS13 activity of > 46% have similar VWF:Ag and VWFpp levels is in disagreement with previous reports showing that an inverse association between ADAMTS13 activity and both markers should exist [16,17]. However, a recent study confirmed our findings, and indicated that the inverse correlation between the two markers does not necessarily exist [18].…”

“…All patients with an acute TTP episode (and ADAMTS13 activity of < 10%) had significantly higher VWF:Ag and VWFpp levels than controls. This is in agreement with previous studies, where plasma levels of VWF:Ag and VWFpp were high during acute vascular disorders, including TTP [5,15,16], suggesting that elevated VWFpp (together with elevated VWF:Ag) is a biomarker of acute endothelial cell activation. We found no difference in levels between patients with a first episode and those with a recurrent episode.…”

Platelet Activation Assessed by Glycoprotein VI/Platelet Ratio Is Associated With Portal Vein Thrombosis After Hepatectomy and Splenectomy in Patients With Liver Cirrhosis

“…Therefore, VWF and VWFpp are considered to be a marker of vascular endothelial cell injury. 34 Virchow triad, which is now frequently applied to describe venous thrombosis, consists of 3 factors: alterations in blood flow, injuries to vascular endothelium, and hypercoagulability. 35 Because we could not measure alterations in portal venous blood flow, we focused on injuries to vascular endothelium which was estimated by plasma levels of VWF and ADAMTS13, and hypercoagulability which was estimated by plasma levels of sGPVI.…”

Plasma ADAMTS13, von Willebrand Factor (VWF), and VWF Propeptide Profiles in Patients With Connective Tissue Diseases and Antiphospholipid Syndrome

“…23 Elevated plasma levels of VWFpp have been reported in patients with thrombotic microangiopathy (TMA) and disseminated intravascular coagulation (DIC). 24,25 In the present study, we measured ADAMTS13 activity and VWFpp and VWF antigen levels in plasma samples from 103 patients with CTD or APS and 68 healthy volunteers (HV).…”