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Severe secondary deficiency of von Willebrand factor–cleaving protease (ADAMTS13) in patients with sepsis-induced disseminated intravascular coagulation: its correlation with development of renal failure
Abstract
Deficiency of ADAMTS13 is found in patients with thrombotic thrombocytopenic purpura (TTP), and the genetic defects in the ADAMTS13 gene or the autoantibody against ADAMTS13 is thought to be responsible for the development of TTP. The clinical correlation and mechanisms of secondary ADAMTS13 deficiency in other disease states were investigated. In addition to TTP, ADAMTS13 levels were severely decreased in patients with sepsis-induced disseminated intravascular coagulation (DIC). The incidence of acute renal failure and serum creatinine levels in patients with ADAMTS13 activity levels lower than 20% (incidence, 41.2%; creatinine, 160 ± 150 μM [1.81 ± 1.70 mg/dL]) (P < .05) were significantly higher than they were in patients with ADAMTS13 activity levels higher than 20% (incidence, 15.4%; creatinine, 84 ± 67 μM [0.95 ± 0.76 mg/dL]) (P < .01). Additionally, unusually large von Willebrand factor multimers were detected in 26 (51.0%) of 51 patients with ADAMTS13 activity levels lower than 20%. Lower molecular weight forms of ADAMTS13 were found in the plasma of patients with sepsis-induced DIC, suggesting that the deficiency of ADAMTS13 was partially caused by its cleavage by proteases in addition to decreased synthesis in the liver. These data suggested that severe secondary ADAMTS13 deficiency can be associated with sepsis-induced DIC and may contribute to the development of renal failure.
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Citations
Cited by
Pierrakos, C., & Vincent, J.-L. (2010) · Critical Care · doi:10.1186/cc8872 · Cited by 1,377
Weiss, S. L., Peters, M. J., Alhazzani, W., Agus, M. S. D., Flori, H. R., Inwald, D. P., Nadel, S., Schlapbach, L. J., Tasker, R. C., Argent, A. C., et al. (2020) · Pediatric Critical Care Medicine · doi:10.1097/pcc.0000000000002198 · Cited by 1,228
Thrombotic thrombocytopenic purpura
Joly, B. S., Coppo, P., & Veyradier, A. (2017) · Blood · doi:10.1182/blood-2016-10-709857 · Cited by 716
Weiss, S. L., Peters, M. J., Alhazzani, W., Agus, M. S. D., Flori, H. R., Inwald, D. P., Nadel, S., Schlapbach, L. J., Tasker, R. C., Argent, A. C., et al. (2020) · Intensive Care Medicine · doi:10.1007/s00134-019-05878-6 · Cited by 606
Thrombotic Thrombocytopenic Purpura
(2006) · New England Journal of Medicine · doi:10.1056/nejmc061483 · Cited by 573
Metadata
- Authors
- Ono, T., Mimuro, J., Madoiwa, S., Soejima, K., Kashiwakura, Y., Ishiwata, A., Takano, K., Ohmori, T., & Sakata, Y.
- Venue
- Blood
- Publisher
- American Society of Hematology
- Published
- 2006-01-15
- Volume
- 107
- Issue
- 2
- Pages
- 528-534
- ISSN
- 0006-4971 (ISSN-L)
- 1528-0020
- OpenAlex
- W2017199698
- Cited by
- 399
- References
- 0
- FWCI
- 7.83
- Type
- journal article
- Language
- en
- Open access
- closed
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- CoChat. (2026). Citation check: Severe secondary deficiency of von Willebrand factor–cleaving protease (ADAMTS13) in patients with sepsis-induced disseminated intravascular coagulation: its correlation with development of renal failure. https://app.cochat.ai/papers/10.1182/blood-2005-03-1087
- The paper
- Ono, T., Mimuro, J., Madoiwa, S., Soejima, K., Kashiwakura, Y., Ishiwata, A., Takano, K., Ohmori, T., & Sakata, Y. (2006). Severe secondary deficiency of von Willebrand factor–cleaving protease (ADAMTS13) in patients with sepsis-induced disseminated intravascular coagulation: its correlation with development of renal failure. Blood. https://doi.org/10.1182/blood-2005-03-1087
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