Clinical Significance of von Willebrand Factor-Cleaving Protease (ADAMTS13) Deficiency in Patients with Sepsis-Induced Disseminated Intravascular Coagulation

Abstract

BACKGROUND: Deficiency of von Willebrand factor-cleaving protease, a disintegrin-like and metalloprotease with thrombospondin type 1 motif 13 (ADAMTS13), is thought to be responsible for platelet aggregation and microthrombi formation, which in turn cause typical thrombotic microangiopathies. This deficiency is found in patients with thrombocytopenia-associated multiple organ failure such as thrombocytopenic purpura and disseminated intravascular coagulation (DIC). We evaluated the clinical significance of ADAMTS13 deficiency in patients with sepsis-induced DIC. MATERIALS AND METHODS: Nineteen patients with sepsis-induced DIC were enrolled. ADAMTS13 antigen levels were determined by Enzyme-Linked Immunosorbent Assay (ELISA) and activity levels were measured by fluorescence resonance energy transfer assay. Patients were categorized into two groups according to ADAMTS13 antigen level: less than 350 ng/mL or above. Clinical characteristics and survival were compared between the two groups. RESULTS: ADAMTS13 antigen level was less than 350 ng/mL in 7 patients and was above 350 ng/mL in 12 patients. There were no significant differences between the groups for age, sex, severity of illness, and other clinical characteristics. In patients with ADAMTS13 antigen level less than 350 ng/mL, in-hospital mortality was much higher (100% versus 25%, P=0.003) and 7-day survival was much shorter (P=0.023). CONCLUSION: Deficiency of ADAMTS13 could be thought to be associated with unfavorable outcome in patients with sepsis-induced DIC.