ArticleAmerican journal of hematology2016
Pulmonary platelet thrombi and vascular pathology in acute chest syndrome in patients with sickle cell disease.
Article in American journal of hematology, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 27 papers.
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Who cites it
27 citing papers in PubMed, 40 citations in OpenAlex.
- Thrombocytosis and the generation of platelet-derived microparticles in the pathophysiology of sickle cell disease.British journal of haematology · 2026Review
- Acute therapy with hemopexin (CSL889) resolves LPS-induced lung vaso-occlusion in sickle mice.Blood vessels, thrombosis & hemostasis · 2026Article
- Proteomic Analysis of Golden Sputum Reveals Pulmonary Complement Activation During Acute Chest Syndrome in Children With Sickle Cell Disease.American journal of hematology · 2026Article
- CD39 polymorphism enables lung thrombosis in sickle cell disease.Nature communications · 2026Article
- Steady-State Ratio of von Willebrand Factor Antigen to ADAMTS13 Activity Predicts Low Platelet Count at Hospitalization for Sickle Cell Disease Vaso-Occlusive Episodes.American journal of hematology · 2025Article
- Respiratory management of acute chest syndrome in children with sickle cell disease.European respiratory review : an official journal of the European Respiratory Society · 2024Review
- End Organ Affection in Sickle Cell Disease.Cells · 2024Review
- The Role of Inflammation in The Cellular and Molecular Mechanisms of Cardiopulmonary Complications of Sickle Cell Disease.Biomolecules · 2023Review
- Thrombo-Inflammation in COVID-19 and Sickle Cell Disease: Two Faces of the Same Coin.Biomedicines · 2023Review
- Article
- Endothelial dysfunction and hypercoagulability in severe sickle-cell acute chest syndrome.ERJ open research · 2021Article
- Sickle cell vaso-occlusion: The dialectic between red cells and white cells.Experimental biology and medicine (Maywood, N.J.) · 2021Review
- COVID-19-induced endotheliitis: emerging evidence and possible therapeutic strategies.British journal of haematology · 2021Article
- Review
- The molecular basis for the prothrombotic state in sickle cell disease.Haematologica · 2020Article
- Sickle Cell Disease: A Paradigm for Venous Thrombosis Pathophysiology.International journal of molecular sciences · 2020Review
- Intravascular hemolysis triggers ADP-mediated generation of platelet-rich thrombi in precapillary pulmonary arterioles.JCI insight · 2020Article
- Tandem P-selectin glycoprotein ligand immunoglobulin prevents lung vaso-occlusion in sickle cell disease mice.Experimental hematology · 2020Article
- Shedding New Light on Platelet Extracellular Vesicles in Sickle Cell Disease.American journal of respiratory and critical care medicine · 2020Article
- Platelet Extracellular Vesicles Drive Inflammasome-IL-1β-Dependent Lung Injury in Sickle Cell Disease.American journal of respiratory and critical care medicine · 2020Article
Corrections and comments
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Authors and funding
8 authors at 2 institutions in 1 country.
Funding
Abstract
A growing body of evidence suggests a role for platelets in sickle cell disease (SCD). Despite the proinflammatory, occlusive nature of platelets, a role for platelets in acute chest syndrome (ACS), however, remains understudied. To provide evidence and potentially describe contributory factors for a putative link between ACS and platelets, we performed an autopsy study of 20 SCD cases-10 of whom died from ACS and 10 whose deaths were not ACS-related. Pulmonary histopathology and case history were collected. We discovered that disseminated pulmonary platelet thrombi were present in 3 out of 10 of cases with ACS, but none of the matched cases without ACS. Those cases with detected thrombi were associated with significant deposition of endothelial vWF and detection of large vWF aggregates adhered to endothelium. Potential clinical risk factors were younger age and higher platelet count at presentation. However, we also noted a sharp and significant decline in platelet count prior to death in each case with platelet thrombi in the lungs. In this study, neither hydroxyurea use nor perimortem transfusion was associated with platelet thrombi. Surprisingly, in all cases, there was profound pulmonary artery remodeling with both thrombotic and proliferative pulmonary plexiform lesions. The severity of remodeling was not associated with a severe history of ACS, or hydroxyurea use, but was inversely correlated with age. We thus provide evidence of undocumented presence of platelet thrombi in cases of fatal ACS and describe clinical correlates. We also provide novel correlates of pulmonary remodeling in SCD.
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