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Comorbidities and Consequences

PNH is a progressive, destructive, and life-threatening disease5,8-10,12

  • 35% of patients with PNH die within 5 years of diagnosis1

Learn more about some of these life-threatening complications by clicking on the links below.

Thrombosis is the leading cause of death in PNH, and even the first thrombotic event can be fatal1-3
64% of patients with PNH have chronic kidney disease (CKD), which in advanced stages is associated with premature mortality4,14
Nearly 50% of patients with PNH have evidence of pulmonary hypertension (PHT)15

Early diagnosis is essential for improved patient management and prognosis. Learn about ICCS and other expert recommendations for who should be tested for PNH.

PNH has been called “the most vicious acquired thrombophilic state known in medicine”. 15 Learn which common symptoms are associated with an increased risk of thromboembolism in a patient diagnosed with PNH.

References: 1. Hillmen P, Lewis SM, Bessler M, et al. N Engl J Med. 1995;333:1253-1258. 2. Socié G, Mary J-Y, de Gramont A, et al; for the French Society of Haematology. Lancet. 1996;348:573-577. 3. Nishimura J-I, Kanakura Y, Ware RE, et al. Medicine. 2004;83:193-207. 4. Hillmen P, Elebute M, Kelly R, et al. Am J Haematol. 2010;85:553-559. 5. Rachidi S, Musallam KM, Taher AT. A closer look at paroxysmal nocturnal hemoglobinuria. Eur J Intern Med. 2010;21:260-267. 6. Helley D, Peffault de Latour R, Porcher R, et al; on behalf of the French Society of Hematology. Haematologica. 2010;95:574-581. 7. Hillmen P, Muus P, Dührsen U, et al. Blood. 2007;110:4123-4128. 8. Brodsky RA. Paroxysmal nocturnal hemoglobinuria. 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. 9. Rother RP, Rollins SA, Mojcik CF, et al. Nat Biotechnol. 2007;25:1256-1264. [Published correction appears in Nat Biotechnol. 2007;25:1488]. 10. Hill A, Sapsford RJ, Scally A, et al. Under-recognized complications in patients with paroxysmal nocturnal haemoglobinuria: raised pulmonary pressure and reduced right ventricular function. Br J Haematol. 2012;158:409-414. 11. Weitz I, Meyers G, Lamy T, et al. Cross-sectional validation study of patient-reported outcomes in patients with paroxysmal nocturnal haemoglobinuria. Intern Med J. 2013;43:298-307. 12. Rother RP, Bell L, Hillmen P, et al. The clinical sequelae of intravascular hemolysis and extracellular plasma hemoglobin: a novel mechanism of human disease. JAMA. 2005;293:1653-1662. 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. Kim JS, Jang JH, Lee JW, et al. Renal impairment is a risk factor for early mortality in patients with paroxysmal nocturnal hemoglobinuria (PNH). In: Posters of the 16th Congress of the European Hematology Association; June 9-12, 2011; London, United Kingdom. Abstract 0271. 15. Hill A, Rother RP, Wang X, et al. Br J Haematol. 2010;149:414-425. 16. Hill A, Kelly R, Hillmen P. Thrombosis in paroxysmal nocturnal hemoglobinuria. Blood. 2013;121:4985-4996. 17. Nishimura J-I, Kanakura Y, Ware RE, et al. Clinical course and flow cytometric analysis of paroxysmal nocturnal hemoglobinuria in the United States and Japan. Medicine. 2004;83:193-207. 18. Socié G, Mary J-Y, de Gramont A, et al; for the French Society of Haematology. Paroxysmal nocturnal haemoglobinuria: long-term follow-up and prognostic factors. Lancet. 1996;348:573-577. 19. Kelly RJ, Hill A, Arnold LM, et al. Blood. 2011;117:6786-6792. 20. Lee JW, Jang JH, Kim JS, et al. Clinical signs and symptoms associated with increased risk for thrombosis in patients with paroxysmal nocturnal hemoglobinuria from a Korean Registry. Int J Hematol. 2013;97:749-757. 21. De Stefano V, Rossi E, Paciaroni K, et al. Screening for inherited thrombophilia: indications and therapeutic implications. Haematologica. 2002;87:1095-1108. 22. McKeage K. Drugs. 2011;71:2327-2345. 23. Weitz IC. Thrombosis in paroxysmal nocturnal hemoglobinuria—insights into the role of complement in thrombosis. Thromb Res. 2010;125:S106-S107.