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Pathophysiology of PNH

The complications from PNH are devastating and systemic. The pathophysiology behind these complications, while complement-mediated, is multifactorial in nature.1-5

PNH has been called “the most vicious acquired thrombophilic state known in medicine”.17 Learn which common symptoms are associated with an increased risk of thromboembolism in a patient diagnosed with PNH.

References: 1. Borowitz MJ, Craig FE, DiGiuseppe JA, et al; for Clinical Cytometry Society. Guidelines for the diagnosis and monitoring of paroxysmal nocturnal hemoglobinuria and related disorders by flow cytometry. Cytometry Part B. 2010;78B:211-230. 2. Richards SJ, Hill A, Hillmen P. Cytometry Part 8. 2007;72B:291-298. 3. Hillmen P, Lewis SM, Bessler M, et al N EngJJ Med. 1995;333:1253- 1258. 4. Brodsky RA. Blood Rev. 2008;.22:65-74. 5. Rachidi S, Musalam KM, Taber AT. E11J Intern Med. 2010;21:260-267. 6. Barac A, Panza JA. Hypertension. 2009;53:900-902. 7. Hillmen P, Elebtrte M, Kelly R, et al AmJ Hematol. 2010;85:553-559. 8. Adams T, Fleischer D, Marino G, et al. Dig Dis Sci. 2002;47:58-64. 9. Brodsky RA. In: Hoffman R, Benz EJ Jr, Shattil SJ, et al, eds. Hematology: Basic Principles and Practice. 4th ed. Philadelphia, PA: Elsevier Churchill Livingstone; 2005:419-427. 10. Kelly R, Richards S, Hillmen P, et al. Ther Clin Risk Manag. 2009;5:911-921. 11. Kim-Shapiro DB, Schechter AN, Gladwin MT. Unraveling the reactions of nitric oxide, nitrite, and hemoglobin in physiology and therapeutics. Arterioscler Thromb Vasc Biol. 2006;26:697-705. 12. Hill A, Rother RP, Wang X, et al. Br J Haematol. 2010;149:414-425. 13. Hill A, Richards SJ, Hillmen P. Recent developments in the understanding and management of paroxysmal nocturnal haemoglobinuria. Br J Haematol. 2007;137:181-192. 14. Bombeli T, Schwartz BR, Harlan JM. Adhesion of activated platelets to endothelial cells: evidence for a GPIIbIIIa-dependent bridging mechanism and novel roles for endothelial intercellular adhesion molecule 1 (ICAM-1), αvβ3 integrin, and GPIbα. J Exp Med. 1998;187:329-339. 15. Majek P, Reicheltova Z, Stikarova J, et al. Proteome Sci. 2010; 8:56 16. Hill A, Sapsford RJ, Scally A, et al. Br J Haematol. 2012;158:409-414. 17. Hill A, Kelly R, Hill P. Blood. 2013;121:4985-4996. 18. Hillmen P, Muus P, Dührsen U, et al. Blood. 2007;110:4123-4128.