ReviewInternational journal of molecular sciences2024
Thrombosis in Paroxysmal Nocturnal Hemoglobinuria (PNH): From Pathogenesis to Treatment.
Review in International journal of molecular sciences, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed.
- Intracranial venous thrombosis imaging by anatomical location: an educational review with mimics and diagnostic pitfalls.Insights into imaging · 2026Article
- Polycythemia Vera, Thrombophilia, CTEPH, Cerebral Venous Sinus Thrombosis and Vertebral Artery Occlusion: A Case-Illustrated Narrative Review of Competing Thrombotic and Hemorrhagic Risks.Life (Basel, Switzerland) · 2026Article
- A case report of CD4Annals of hematology · 2026Article
- Clonal Hematopoiesis of Indeterminate Potential (CHIP): A Model of Mutation-Driven Thromboinflammation.Cancers · 2026Review
- Thrombin generation in PNH patients treated sequentially with Eculizumab and Ravulizumab: a paired analysis.Journal of thrombosis and thrombolysis · 2026Article
- Beyond Terminal Blockade: A Mechanism-Based Approach to Complement Inhibitor Selection in Paroxysmal Nocturnal Hemoglobinuria.Drug design, development and therapy · 2026Review
- MUC4 mutations promote a thrombotic phenotype in patients with paroxysmal nocturnal haemoglobinuria by increasing the deposition of terminal complement.Clinical and translational medicine · 2026Article
- A real-world, multi-center, prospective, observational study for paroxysmal nocturnal haemoglobinuria (PNH) in China: Baseline characteristics, disease burden and treatment patterns.Therapeutic advances in hematology · 2026Article
- Editorial: Endothelium, innate immunity and coagulation in hematological disorders.Frontiers in medicine · 2026Article
- Paroxysmal Nocturnal Hemoglobinuria-Driven Hepatic Vein Thrombosis: A Case of Budd-Chiari Syndrome in Disguise.Cureus · 2025Article
- Activated Complement System's Impact in Antiphospholipid Syndrome Thrombosis: From Pathophysiology to Treatment.Journal of clinical medicine · 2025Review
- Paroxysmal Nocturnal Hemoglobinuria: Unraveling Its Molecular Pathogenesis and Advancing Targeted Therapeutic Strategies.Diseases (Basel, Switzerland) · 2025Review
- Common Genetic Variants in Rare Disorders: Hematology and Beyond.Current issues in molecular biology · 2025Article
- Clinical impact of quality nursing combined with health education pathway on quality of life and sleep in severe aplastic anemia patients complicated with infection: a randomized clinical trial.Frontiers in medicine · 2025Article
- Paroxysmal nocturnal hemoglobinuria masquerading as hemolytic uremic syndrome: a Case Report.Frontiers in medicine · 2025Article
- Mixed-type autoimmune hemolytic anaemia complicated by acute cerebral infarction: a case report.Frontiers in medicine · 2025Article
- Smart Thrombosis Care: The Rise of Closed-Loop Diagnosis-to-Treatment Nano Systems.International journal of nanomedicine · 2025Review
Corrections and comments
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Authors and funding
9 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Paroxysmal Nocturnal Hemoglobinuria (PNH) constitutes a rare bone marrow failure syndrome characterized by hemolytic anemia, thrombotic events (TEs), and bone marrow aplasia of variable degrees. Thrombosis is one of the major clinical manifestations of the disease, affecting up to 40% of individuals with PNH. Venous thrombosis is more prevalent, affecting mainly unusual sites, such as intrabdominal and hepatic veins. TEs might be the first clinical manifestation of PNH. Complement activation, endothelial dysfunction, hemolysis, impaired bioavailability of nitric oxide, and activation of platelets and neutrophils are implicated in the pathogenesis of TEs in PNH patients. Moreover, a vicious cycle involving the coagulation cascade, complement system, and inflammation cytokines, such as interleukin-6, is established. Complement inhibitors, such as eculizumab and ravulizumab (C5 inhibitors), have revolutionized the care of patients with PNH. C5 inhibitors should be initiated in patients with PNH and thrombosis, while they constitute a great prophylactic measure for TEs in those individuals. Anticoagulants, such as warfarin and low-molecular-weight heparin, and, in selected cases, direct oral anticoagulants (DOACs) should be used in combination with C5 inhibitors in patients who develop TEs. Novel complement inhibitors are considered an alternative treatment option, especially for those who develop extravascular or breakthrough hemolysis when terminal inhibitors are administered.
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