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Original Articles
Clinical Significance of von Willebrand Factor-Cleaving Protease (ADAMTS13) Deficiency in Patients with Sepsis-Induced Disseminated Intravascular Coagulation
Young Keun Kim, M.D.1, Jongwook Lee, M.D.2, Kyung-A Lee, M.D.3 and Hee Uk Kwon, M.D.4
Department of Internal Medicine1, Yonsei University Wonju College of Medicine, Wonju, Department of Laboratory, Medicine2, Hanaro Medical Foundation, Seoul, Department of Laboratory Medicine3, Yonsei University College of Medicine, Seoul, Department of Anesthesiology and Pain Medicine4, Konyang University College of Medicine, Daejeon, Korea
Vol.41 Num.2 (p78~80)
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.
Keywords : ADAMTS13, Disseminated intravascular coagulation, Sepsis, Prognosis