Article
Interrelationship between ADAMTS13 Activity, von Willebrand Factor, and Complement Activation in Remission from Immune-Mediated Thrombotic Thrombocytopenic Purpura.
British Journal of Haematology
(2020)
Abstract
Severely deficient ADAMTS13 activity (<10%) is an important risk factor to predict relapse in immune-mediated thrombotic thrombocytopenic purpura (iTTP) (Ferrari et al., 2007; Jin et al., 2008; Peyvandi et al., 2008). It is also presumed that the in vitro ADAMTS13 activity correlates with in vivo function as defined by von Willebrand factor (VWF) multimers. Activation of complement plays a role in the pathophysiology of iTTP (Reti et al., 2012; Wu et al., 2013; Feng et al., 2015; Turner et al., 2015). Published data suggest that the ULVWF multimers serve as a scaffold for the activation of the alternative complement pathway, and less efficiently inhibit complement activation compared to physiologic sized VWF multimers (Feng et al., 2015; Turner et al., 2015). Complement activation has been demonstrated to be increased in acute iTTP episodes (Reti et al., 2012) and be associated with an increased mortality rate (Wu et al., 2013). We analyzed the ADAMTS13 biomarker data, VWF multimer status, and complement activation biomarker data in banked samples obtained during clinical remission in patients with a diagnosis of iTTP.
Disciplines
Publication Date
February 17, 2020
Citation Information
Camila Masias. "Interrelationship between ADAMTS13 Activity, von Willebrand Factor, and Complement Activation in Remission from Immune-Mediated Thrombotic Thrombocytopenic Purpura." British Journal of Haematology (2020) Available at: http://works.bepress.com/camila-masiascastanon/25/