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Clinical Manifestations

Chronic complement-mediated hemolysis leads to devestating consequences.1,2

In PNH, certain outward symptoms can be indicative of underlying, life-threatening complications. Therapeutic intervention should be considered if a patient exhibits any of these common symptoms, which include2,16,18,20:

  • Abdominal pain
  • Chest pain
  • Dyspnea
  • Hemoglobinuria
  • Dysphagia
  • Erectile dysfunction
  • Fatigue

A comprehensive clinical assessment is crucial to determine your PNH patient’s risk of morbidities and premature mortality.3-5,17,18

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References: 1. Rachidi S, Musallam KM, Taher AT. A closer look at paroxysmal nocturnal hemoglobinuria. Eur J Intern Med. 2010;21:260-267. 2. Borowitz MJ, Craig FE, DiGiuseppe JA, et al; for Clinical Cytometry Society. Cytometry Part B. 2010;78B:211-230. 3. Hill A, Richards SJ, Hillmen P. Recent developments in the understanding and management of paroxysmal nocturnal haemoglobinuria. Br J Haematol. 2007;137:181-192. 4. Parker C, Omine M, Richards S, et al; for International PNH Interest Group. Diagnosis and management of paroxysmal nocturnal hemoglobinuria. Blood. 2005;106:3699-3709. 5. Hill A, Rother RP, Wang X, et al. Br J Haematol. 2010;149:414-425. 6. Hillmen P, Muus P, Dührsen U, et al. Blood. 2007;110:4123-4128. 7. McKeage K. Drugs. 2011;71:2327-2345. 8. 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. 9. De Cobelli F, Pezzetti G, Margari S, et al. New insights in abdominal pain in paroxysmal nocturnal hemoglobinuria (PNH): a MRI study. PLOS ONE. 2014;10:e0122832. 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. Brodsky A, Mazzocchi O, Sanchez F, et al. Exp Hematol Oncol. 2012;1:26. 12. Hillmen P, Muus P, Röth A, et al. Br J Haematol. 2013;162:62-73. 13. Clark DA, Butler SA, Braren V, et al. The kidneys in paroxysmal nocturnal hemoglobinuria. Blood. 1981;57:83-89. 14. Nair RK, Khaira A, Sharma A, et al. Spectrum of renal involvement in paroxysmal nocturnal hemoglobinuria: report of three cases and a brief review of the literature. Int Urol Nephrol. 2008;40:471-475. 15. Muñoz-Linares C, Ojeda E, Forés R, et al. Paroxysmal nocturnal hemoglobinuria: a single Spanish center’s experience over the last 40 yr. Eur J Haematol. 2014;93:309-319. 16. Schrezenmeier H, Muus P, Socié G, et al. Baseline characteristics and disease burden in patients in the international paroxysmal nocturnal hemoglobinuria registry. Haematologica. 2014;99:922-929. 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. 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. 19. Dacie JV, Lewis SM. Paroxysmal nocturnal haemoglobinuria: clinical manifestations, haematology, and nature of the disease. Ser Haemat. 1972;5:3-23. 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. 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.