ADAMTS13: Structure and Function

Zheng, X. Long

doi:10.1007/978-3-319-08717-7_3

Cited by 1 publication

(1 citation statement)

References 121 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…ADAMTS‐13, a plasma metalloprotease, plays a critical role in regulating hemostasis and preventing unwanted thrombosis. 26 It does so through proteolytic cleavage of von Willebrand factor (VWF), which is synthesized and released from activated or injured endothelium. 27 Severe deficiency of plasma ADAMTS‐13, primarily resulting from acquired autoantibodies against ADAMTS‐13, leads to thrombotic thrombocytopenic purpura (TTP), a potentially fatal blood disorder.…”

Section: Introductionmentioning

confidence: 99%

Low ADAMTS‐13 predicts adverse outcomes in hospitalized patients with suspected heparin‐induced thrombocytopenia

Chan

Zhao

Zheng

2021

Research and Practice in Thrombosis and Haemostasis

Self Cite

View full text Add to dashboard Cite

Background Heparin‐induced thrombocytopenia (HIT) is a life‐threatening thrombotic complication after heparin exposure. However, the role of ADAMTS‐13 and von Willebrand factor (VWF) in the disease process and outcomes of HIT is not known. Objective To determine the potential role of ADAMTS‐13 and VWF in hospitalized patients suspected with HIT. Methods Associations of the HIT tests, ADAMTS‐13 activity, and VWF antigen or activity with other clinical parameters and outcomes in the patients suspected with HIT were determined. Results Of 261 patients, 87 (33.3%) were positive and 174 (66.7%) were negative for a HIT antibody determined by an enzyme immunoassay (EIA). Of these 87 EIA+ patients, 31 (35.6%) were also positive but 56 (64.4%) were negative for serotonin‐releasing assay (SRA). There was no statistically significant difference among all three groups (i.e., EIA–, EIA+/SRA+, and EIA+/SRA–) as to their demographic features, reasons for admission to the hospital, type of procedures performed, and in‐hospital mortality. Compared to those in the healthy controls, plasma ADAMTS‐13 activity in patients suspected with HIT was significantly lower but plasma VWF antigen (VWFAg) and activity (VWFAc) in these patients were significantly higher. While there was no statistically significant difference among all three groups regarding plasma levels of ADAMTS‐13 activity, VWFAg, and VWFAc, plasma levels of ADAMTS‐13 activity <50% or the low ratios of ADAMTS‐13 activity to VWFAg (or VWFAc) are highly predictive for a 90‐day mortality rate, particularly in the EIA+SRA+ group. Conclusions These results demonstrate that relative deficiency of plasma ADAMTS‐13 activity in hospitalized patients suspected with HIT is common, which may contribute at least in part to the adverse outcomes in this patient population, particularly in those with true HIT.

show abstract

Section: Introductionmentioning

confidence: 99%