ArticleHaematologica2008
Coagulation activation and inflammation in sickle cell disease-associated pulmonary hypertension.
Article in Haematologica, 2008. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 84 papers, 2 of them syntheses that pooled it.
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The trial behind it
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Who cites it
84 citing papers in PubMed, 2 syntheses or guidelines pooled it, 185 citations in OpenAlex.
- Pooled it
- An official American Thoracic Society clinical practice guideline: diagnosis, risk stratification, and management of pulmonary hypertension of sickle cell disease.American journal of respiratory and critical care medicine · 2014Guideline
- Oral tetrahydrouridine and decitabine for non-cytotoxic epigenetic gene regulation in sickle cell disease: A randomized phase 1 study.PLoS medicine · 2017Trial
- Trial
- A double-blind, randomized, multicenter phase 2 study of prasugrel versus placebo in adult patients with sickle cell disease.Journal of hematology & oncology · 2013Trial
- Elevated tricuspid regurgitation velocity in adults with sickle cell disease and HbAA controls in Kinshasa, Democratic Republic of the Congo: a comparative cross-sectional study.Annals of hematology · 2026Article
- Hydroxyurea in Sickle Cell Disease: Coagulation and Activation in an Observational Study.Clinical medicine insights. Pediatrics · 2026Article
- Abnormalities of hemostasis in sickle cell patients and predisposition to thrombotic risk: a systematic review and meta-analysis.Thrombosis journal · 2025Review
- Genetic variants associated with white blood cell count amongst individuals with sickle cell disease.British journal of haematology · 2024Article
- Non-invasive optical and laboratory hematologic biomarkers correlate in patients with sickle cell disease.Biomedical optics express · 2024Article
- End Organ Affection in Sickle Cell Disease.Cells · 2024Review
- Assessment of hypoxemia among young adults with sickle cell anaemia in steady state in southwestern Nigeria: a crosssectional study.BMC research notes · 2024Article
- The Role of Inflammation in The Cellular and Molecular Mechanisms of Cardiopulmonary Complications of Sickle Cell Disease.Biomolecules · 2023Review
- Extracellular Vesicles in Sickle Cell Disease: A Promising Tool.Bioengineering (Basel, Switzerland) · 2022Review
- Incidence of Deep Venous Thrombosis and Sickle Cell Disease in Patients Undergoing Spinal Surgery in South Gujarat, India: A Prospective Observational Study.Malaysian orthopaedic journal · 2022Article
- Assessment of Reticulocyte and Erythrocyte Parameters From Automated Blood Counts in Vaso-Occlusive Crisis on Sickle Cell Disease.Frontiers in medicine · 2022Article
- The different facets of sickle cell disease-related pulmonary hypertension.Current opinion in pulmonary medicine · 2021Review
- Tripartite collaboration of blood-derived endothelial cells, next generation RNA sequencing and bioengineered vessel-chip may distinguish vasculopathy and thrombosis among sickle cell disease patients.Bioengineering & translational medicine · 2021Article
- Increased hemoglobin affinity for oxygen with GBT1118 improves hypoxia tolerance in sickle cell mice.American journal of physiology. Heart and circulatory physiology · 2021Article
- Extracellular Vesicles in Sickle Cell Disease: Plasma Concentration, Blood Cell Types Origin Distribution and Biological Properties.Frontiers in medicine · 2021Review
24 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
10 authors at 4 institutions in 2 countries.
Funding
Abstract
backgroundPulmonary hypertension (PHT) is common in sickle cell disease (SCD). The purpose of this study was to determine whether markers of coagulation activation and inflammation are associated with PHT in SCD. DESIGN AND
methodsThis cross-sectional study was performed using a cohort of patients followed at an adult Sickle Cell Clinic. Pulmonary artery systolic pressure was determined by Doppler echocardiography, and the diagnosis of PHT was defined using age, sex and body mass index-adjusted reference ranges. Clinical laboratory examinations, including hematologic studies and biochemical tests, as well as various measures of coagulation activation, endothelial activation and inflammation, were conducted on SCD subjects and on healthy, race-matched control subjects without SCD.
resultsPatients with SCD (n=76) had higher plasma levels of markers of coagulation (thrombin-antithrombin complex, prothrombin fragment F1+2, D-dimer) and endothelial (soluble vascular endothelial cell adhesion molecule, sVCAM) activation compared with control subjects (n=6). SCD patients with PHT (n=26) had significantly higher levels of sVCAM compared with those patients without PHT (n=50). Although PHT patients showed increased plasma measures of coagulation activation, the differences were not statistically significant when compared to those of patients without PHT. HbSS patients with PHT also had a trend towards higher levels of other inflammatory cytokines (interleukins 6, 8 and 10) than HbSS patients without PHT. There was a modest negative correlation between hemoglobin and plasma measures of coagulation and endothelial activation, and modest positive correlations between markers of hemolysis and plasma measures of coagulation and endothelial activation.
conclusionsSCD patients with PHT have higher levels of markers of endothelial activation and other inflammatory markers than patients without PHT. A trend towards an increased level of markers of coagulation activation was observed in SCD patients with PHT compared with that in patients without PHT. Markers of hemolysis are associated with coagulation activation and endothelial dysfunction in SCD patients. Clinical trials of anticoagulants and anti-inflammatory agents are warranted in SCD patients with PHT.
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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.