Prognostic value of the ADAMTS13-vWF axis in disseminated intravascular coagulation: Platelet count/vWF:Ag ratio as a strong prognostic marker.

Abstract

ADAMTS13 deficiency increases the circulating level of von Willebrand factor (vWF). Low ADAMTS13 and high vWF can provide a milieu for microthrombosis, including disseminated intravascular coagulation (DIC). This study investigated the prognostic values of ADAMTS13-vWF axis markers and their correlation with DIC severity. ADAMTS13-vWF axis markers (vWF antigen (vWF:Ag), vWF ristocetin cofactor (vWF:Rco), ADAMTS13 activity, and anti-ADAMTS13 antibody) were measured in patients (n=152) suspected of having DIC along with the well-known DIC markers including antithrombin and protein C. The vWF:Ag level was significantly increased, and ADAMTS13 activity was significantly decreased in overt DIC. The vWF:Ag level (hazard ratio 7.365, p=.009), ADAMTS13 activity/vWF:Ag ratio (hazard ratio 3.777, p=.037), ADAMTS13 activity/vWF:Rco ratio (hazard ratio 3.027, p=.028), and platelet count/vWF:Ag ratio (hazard ratio 8.538, p<.001) were significant prognostic markers in Cox regression analysis and correlated well with DIC score and antithrombin and protein C levels. The platelet count/vWF:Ag was the strongest prognostic marker among ADAMTS13-vWF axis markers. The measurement of vWF:Ag may improve prognostic insights of DIC in clinical practice.