ArticleHaematologica2010
Evaluation of hemostasis and endothelial function in patients with paroxysmal nocturnal hemoglobinuria receiving eculizumab.
Article in Haematologica, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 35 papers.
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Who cites it
35 citing papers in PubMed, 97 citations in OpenAlex.
- Long-term efficacy and safety of eculizumab in Japanese patients with PNH: AEGIS trial.International journal of hematology · 2013Trial
- Platelet count kinetics following interruption of antiretroviral treatment.AIDS (London, England) · 2013Trial
- Allogeneic stem cell transplantation in paroxysmal nocturnal hemoglobinuria.Haematologica · 2012Trial
- Safety and efficacy of the terminal complement inhibitor eculizumab in Japanese patients with paroxysmal nocturnal hemoglobinuria: the AEGIS clinical trial.International journal of hematology · 2011Trial
- Translational immunothrombosis in autoimmune Heparin-Induced thrombocytopenia: targeting the FcγRIIa-Syk-BTK and complement pathways.Clinical and experimental medicine · 2026Review
- Prevention and Management of Thromboembolism in Patients with Paroxysmal Nocturnal Hemoglobinuria in Asia: A Narrative Review.International journal of molecular sciences · 2025Review
- Thrombosis in Paroxysmal Nocturnal Hemoglobinuria (PNH): From Pathogenesis to Treatment.International journal of molecular sciences · 2024Review
- Characteristics and outcomes associated with venous thrombotic complications among patients with paroxysmal nocturnal hemoglobinuria.Annals of hematology · 2023Article
- Circulating Endothelial Progenitor Cells and Their Relation to Thrombosis in Paroxysmal Nocturnal Hemoglobinuria and Aplastic Anemia.Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion · 2022Article
- A 15-year, single institution experience of anticoagulation management in paroxysmal nocturnal hemoglobinuria patients on terminal complement inhibition with history of thromboembolism.American journal of hematology · 2022Article
- How we('ll) treat paroxysmal nocturnal haemoglobinuria: diving into the future.British journal of haematology · 2022Review
- Clinical characteristics and therapeutic outcomes of paroxysmal nocturnal hemoglobinuria patients in Turkey: a multicenter experience.Annals of hematology · 2021Article
- Markers of Thrombin Generation and Inflammation in Patients with Paroxysmal Nocturnal Hemoglobinuria.Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion · 2021Article
- The interaction between the complement system and hemostatic factors.Current opinion in hematology · 2020Review
- Evaluation of endothelial microparticles as a prognostic marker in hemolytic disease of the newborn in China.The Journal of international medical research · 2019Article
- Polymorphism of theOncotarget · 2017Article
- Extracellular vesicles in renal disease.Nature reviews. Nephrology · 2017Review
- Hematological disorders and pulmonary hypertension.World journal of cardiology · 2016Review
- Eculizumab in the management of paroxysmal nocturnal hemoglobinuria: patient selection and special considerations.Therapeutics and clinical risk management · 2016Review
- Recent advances in the pathogenesis and treatment of paroxysmal nocturnal hemoglobinuria.F1000Research · 2016Review
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Authors and funding
11 authors at 5 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundParoxysmal nocturnal hemoglobinuria (PNH) is associated with an increased risk of thrombosis through unknown mechanisms. DESIGN AND
methodsWe studied 23 patients with PNH, before and after five and 11 weeks of treatment with eculizumab. We examined markers of thrombin generation and reactional fibrinolysis (prothrombin fragment 1+2 (F1+2), D-dimers, and plasmin antiplasmin complexes (P-AP), and endothelial dysfunction tissue plasminogen activator (t-PA), plasminogen activator inhibitor (PAI-1), soluble thrombomodulin (sTM), intercellular adhesion molecule 1 (sICAM-1), vascular cell adhesion molecule (sVCAM-1), endothelial microparticles (EMPs), and tissue factor pathway inhibitor (TFPI).
resultsAt baseline, vWF, sVCAM-1, the EMP count, and F1+2 and D-dimer levels were significantly elevated in the patients, including those with no history of clinical thrombosis. Treatment with eculizumab was associated with significant decreases in plasma markers of coagulation activation (F1+2, P=0.012, and D-dimers, P=0.01), and reactional fibrinolysis (P-AP, P=0.0002). Eculizumab treatment also significantly reduced plasma markers of endothelial cell activation (t-PA, P=0.0005, sVCAM-1, P<0.0001, and vWF, P=0.0047) and total (P=0.0008) and free (P=0.0013) TFPI plasma levels.
conclusionsOur results suggest a new understanding of the contribution of endothelial cell activation to the pathogenesis of thrombosis in PNH. The terminal complement inhibitor, eculizumab, induced a significant and sustained decrease in the activation of both the plasma hemostatic system and the vascular endothelium, likely contributing to the protective effect of eculizumab on thrombosis in this setting.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.