The DOI matches its Crossref record; 95 of the 95 references it cites were found in a registry or an index.
Thrombosis in paroxysmal nocturnal hemoglobinuria
Abstract
The most frequent and feared complication of paroxysmal nocturnal hemoglobinuria (PNH) is thrombosis. Recent research has demonstrated that the complement and coagulation systems are closely integrated with each influencing the activity of the other to the extent that thrombin itself has recently been shown to activate the alternative pathway of complement. This may explain some of the complexity of the thrombosis in PNH. In this review, the recent changes in our understanding of the pathophysiology of thrombosis in PNH, as well as the treatment of thrombosis, will be discussed. Mechanisms explored include platelet activation, toxicity of free hemoglobin, nitric oxide depletion, absence of other glycosylphosphatidylinositol-linked proteins such as urokinase-type plasminogen activator receptor and endothelial dysfunction. Complement inhibition with eculizumab has a dramatic effect in PNH and has a major impact in the prevention of thrombosis as well as its management in this disease.
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The Complement Inhibitor Eculizumab in Paroxysmal Nocturnal Hemoglobinuria
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Self-citationReciprocal coupling of coagulation and innate immunity via neutrophil serine proteases
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Cited by
Paroxysmal nocturnal hemoglobinuria
Brodsky, R. A. (2014) · Blood · doi:10.1182/blood-2014-02-522128 · Cited by 648
Paroxysmal nocturnal haemoglobinuria
Hill, A., DeZern, A. E., Kinoshita, T., & Brodsky, R. A. (2017) · Nature Reviews Disease Primers · doi:10.1038/nrdp.2017.28 · Cited by 448
Self-citationKulasekararaj, A. G., Hill, A., Rottinghaus, S. T., Langemeijer, S., Wells, R., Gonzalez-Fernandez, F. A., Gaya, A., Lee, J. W., Gutierrez, E. O., Piatek, C. I., et al. (2019) · Blood · doi:10.1182/blood-2018-09-876805 · Cited by 382
Self-citationOxidative stress, eryptosis and anemia: a pivotal mechanistic nexus in systemic diseases
Bissinger, R., Bhuyan, A. A. M., Qadri, S. M., & Lang, F. (2019) · The FEBS Journal · doi:10.1111/febs.14606 · Cited by 239
Diseases of complement dysregulation-an overview
Wong, E. K. S., & Kavanagh, D. (2018) · Seminars in Immunopathology · doi:10.1007/s00281-017-0663-8 · Cited by 115
Metadata
- Authors
- Hill, A., Kelly, R. J., & Hillmen, P.
- Venue
- Blood
- Publisher
- American Society of Hematology
- Published
- 2013-06-20
- Volume
- 121
- Issue
- 25
- Pages
- 4985-4996
- ISSN
- 0006-4971 (ISSN-L)
- 1528-0020
- OpenAlex
- W2151709211
- Cited by
- 478
- References
- 95
- FWCI
- 17.62
- Type
- journal article
- Language
- en
- Open access
- closed
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