ArticleResearch and practice in thrombosis and haemostasis2026
Monitoring ADAMTS-13 conformation in immune-mediated thrombotic thrombocytopenic purpura: toward personalized management.
Article in Research and practice in thrombosis and haemostasis, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Background: Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a life-threatening thrombotic microangiopathy caused by an autoimmune-driven deficiency of ADAMTS-13. Despite remission, relapses remain a major concern for patients and are currently predicted by monitoring ADAMTS-13 activity. Objectives: This study evaluated the association between ADAMTS-13 conformation and relapse risk in patients with iTTP during follow-up. Methods: We conducted a retrospective monocentric study involving patients with iTTP with ADAMTS-13 monitoring from 2008 to 2020. ADAMTS-13 antigen and conformation were assessed in plasma samples using our 3H9-ELISA and 1C4-ELISA, respectively. Results: Fifteen patients with iTTP were monitored for a median of 7 years (IQR, 6-11) with a total of 479 plasma samples. Based on annual relapse rate (RR; median, 0.5), they were categorized as low (group 1; RR, <0.50, Conclusion: Persistent open ADAMTS-13 conformation in remission samples was observed more frequently in patients with higher relapse risk and could potentially serve as a biomarker for detecting low levels of circulating autoantibodies. This potential biomarker requires prospective validation before it can be used to guide individualized iTTP management.
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