ArticleResearch and practice in thrombosis and haemostasis2021
ADAMTS13 and von Willebrand factor assessment in steady state and acute vaso-occlusive crisis of sickle cell disease.
Article in Research and practice in thrombosis and haemostasis, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed, 18 citations in OpenAlex.
- TTP-Like Syndrome and Subsequent Non-Aneurysmal Subarachnoid Hemorrhage in HbSC Disease: A Case Report.Clinical case reports · 2026Article
- Biomarkers for the diagnosis and phenotyping of acute chest syndrome in patients with sickle cell disease.Expert review of respiratory medicine · 2026Review
- Elevated VWF with normal ADAMTS13 in pediatric sickle cell disease: links to crises and ischemic stroke.Blood vessels, thrombosis & hemostasis · 2026Article
- Thrombotic microangiopathic anemia in patients with sickle cell disease and its variants during a vaso-occlusive crisis.Research and practice in thrombosis and haemostasis · 2025Article
- The Highs and Lows of ADAMTS13 Activity.Journal of clinical medicine · 2024Review
- Prospective study of complement activation and thromboinflammation within sickle cell disease and its complications.HemaSphere · 2024Article
- VWF-ADAMTS13 axis dysfunction in children with sickle cell disease treated with hydroxycarbamide vs blood transfusion.Blood advances · 2023Article
- Targeting the von Willebrand Factor-ADAMTS-13 axis in sickle cell disease.Journal of thrombosis and haemostasis : JTH · 2023Article
- Pitfalls in Diagnosing Thrombotic Thrombocytopenic Purpura in Sickle Cell Disease.Journal of clinical medicine · 2022Article
- Evidence of protective effects of recombinant ADAMTS13 in a humanized model of sickle cell disease.Haematologica · 2022Article
- Contribution of ADAMTS13-independent VWF regulation in sickle cell disease.Journal of thrombosis and haemostasis : JTH · 2022Article
- ADAMTS13 and von Willebrand factor assessment in steady state and acute vaso-occlusive crisis of sickle cell disease.Research and practice in thrombosis and haemostasis · 2021Article
Corrections and comments
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Authors and funding
12 authors at 5 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundSickle cell disease (SCD) is characterized by vaso-occlusive crisis (VOC), acute chest syndrome (ACS) and multiorgan failure (MOF) complicated by thrombosis. Von Willebrand factor (VWF) is a strong marker of SCD-related endothelial injury.
objectivesTo decipher the role of VWF and its specific-cleaving metalloprotease, ADAMTS13, in the vaso-occlusive and thrombotic process of SCD. PATIENTS/
methodsWe investigated the VWF antigen (Ag), ADAMTS13 activity, ADAMTS13 Ag and ADAMTS13 IgGs in a cohort of 65 patients with SCD prospectively enrolled in a 20-month period from three centers. Patients were divided into two groups: an asymptomatic group (n = 30) with treated or untreated SCD at steady state, and a VOC/ACS group (n = 35) with SCD with VOC/ACS requiring either medical management or intensive care management for MOF. RESULTS AND
conclusionsVWF:Ag levels were increased (median, 167 IU/dL; interquartile range [IQR], 124 - 279), especially in patients with VOC SCD (227 IU/dL; IQR, 134-305;
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