Introduction: In disseminated intravascular coagulation (DIC), widespread activation of intravascular coagulation accompanied with florid endothelial activation results in release of unusually large von Willebrand factor (ULvWF) from endothelium. Circulating a disintegrin-like and metalloprotease with thrombospondin type 1 repeats (ADAMTS)-13 may be consumed through the ongoing cleavage of ULvWF, resulting in a secondary deficiency of ADAMTS-13 in DIC. We determined whether ADAMTS-13 activity showed a significant correlation with the activation status of the coagulation system and hospital mortality in DIC.
Materials and methods: ADAMTS-13 activity was assayed by fluorescence resonance energy transfer assay in 97 patients who were clinically suspected to have DIC.
Results: ADAMTS-13 activity gradually decreased based on the DIC score and D-dimer levels and was correlated with the antithrombin level, representing the consumption of ADAMTS-13 during the ongoing coagulation process. There were no correlation between ADAMTS-13 activity and neutrophil CD64 expression as a neutrophil activation marker and circulating IL-6 level as an inflammatory marker. Patients with a low activity of ADAMTS-13 (< or = 56.4%) had a poor survival rate compared to patients with a high activity of ADAMTS-13.
Conclusions: We conclude that ADAMTS-13 activity is strongly correlated with the severity of coagulopathy and hospital mortality. ADAMTS-13 may serve as a diagnostic and prognostic marker of DIC.