Correlation between plasma activity of ADAMTS-13 and coagulopathy, and prognosis in disseminated intravascular coagulation.
BACKGROUND
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.


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.
Hyun
Jungwon
J
Department of Laboratory Medicine, Seoul National University College of Medicine, Seoul, Republic of Korea.
Kim
Hyun Kyung
HK
Kim
Ji-Eun
JE
Lim
Min-Gyu
MG
Jung
Jae Seol
JS
Park
Seonyang
S
Cho
Han-Ik
HI
D051722:ADAM Proteins / Q000097:blood*; D000071120:ADAMTS13 Protein; D000368:Aged; D000991:Antithrombins / Q000032:analysis; D004211:Disseminated Intravascular Coagulation / Q000097:blood* / Q000175:diagnosis / Q000401:mortality*; D004730:Endothelium, Vascular / Q000378:metabolism; D005260:Female; D005338:Fibrin Fibrinogen Degradation Products / Q000032:analysis; D031541:Fluorescence Resonance Energy Transfer; D006801:Humans; D053208:Kaplan-Meier Estimate; D007723:Korea; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012720:Severity of Illness Index; D016019:Survival Analysis; D015996:Survival Rate; D014841:von Willebrand Factor / Q000378:metabolism
D000991:Antithrombins; D005338:Fibrin Fibrinogen Degradation Products; C036309:fibrin fragment D; D014841:von Willebrand Factor; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human