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

Doctor Goldberg video

Watch Jack Goldberg, MD, discuss the limitations of anticoagulation therapy in treating thrombosis in PNH.

Clinical Assessment for Risks of Early Mortality

For more information about the important role of a full clinical assessment for your patient with PNH, download the Clinical Assessment for Risks of Early Mortality.


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.