Blood: Open Conformation of ADAMTS13 - New Biomarkers of Subclinical iTTP

Recently, Roose et alfound that adamTS13 circulated with open conformation altruism during the acute period of immune-mediated thrombocytopenia reduced cyanosis (iTTP)Although the cause of acute iTTP conformational change is still unclear, in patients with iTTP after remission and post-rituximab therapy, ADAMTS13 is mainly present with closed conformation, and in itadays itisses, ADAMTS13 activity is 50%, and the anti-ADAMTS13 self-antibody detection is not detected, indicating the effect of anti-ADAMTS13 self-antibodythe researchers purified IgG from 18 acute iTTP patients and added it to the closed ADAMTS13 in the normal donor plasmaThis led to the emergence of open ADAMTS13 in 14/18 (78%) samples, which proved that anti-ADAMTS13 autoantibodies can induce open ADAMTS13 conformationTo further clarify the conformation of ADAMTS13 in iTTP patients, the researchers studied a new iTTP patient queue (197 people), which also included plasma samples from adamTS13 active, 50% of iTTTP patients in the remission periodS13 conformation is found not only in patients with acute iTTP, but also in patients with ADAMTS13 activity in the remission period, 50% of the patients and half of the ADAMTS13 active 50%, although free anti-ADAMTS13 antibodies are not always detectedtherefore, the opening of ADAMTS13 is not only a marker of acute iTTP, but also a new biomarker for the detection of subclinical iTTP in patients in remissionFinally, a long-term follow-up study of iTTP patients showed that open conformation indicates a serious decline in ADAMTS13 activityin general, this study proves that anti-ADAMTS13 antiantibodies in iTTP patients can induce open ADAMTS13 conformationMost importantly, open ADAMTS13 conformation is a biomarker of subclinical iTTP and can be an important tool for TTP management