Consequences of Hemolysis

Chronic, complement-mediated intravascular hemolysis is the underlying cause of progressive morbidities and mortality in PNH1

  • 35% of PNH patients die within 5 years of diagnosis, despite best historical care2
  • Even in the absence of symptoms, intravascular hemolysis is ongoing and destructive3,4
  • The consequences of chronic intravascular hemolysis can be unpredictable, sudden, and potentially fatal5
  • Plasma-free hemoglobin is toxic and can result in systemic damage to multiple organs6-10
  • Hemolysis-induced nitric oxide depletion results in pulmonary hypertension, abdominal pain, dysphagia, and erectile dysfunction6

The diverse and common symptomatology of PNH can delay diagnosis

A diagnosis of PNH is typically delayed from 1 to more than 10 years.19 The name of the disease can be misleading, as nearly three-fourths of patients with PNH do not present with hemoglobinuria.17 The wide range of symptoms caused by PNH is shown in the chart below.

The diverse symptomatology of PNH drives the need for a comprehensive clinical assessment and routine diagnostic approach.3,17

Watch Eloy Roman, MD, describe the devastating clinical manifestations caused by nitric oxide depletion due to intravascular hemolysis.

Early diagnosis can positively impact long-term patient outcomes. Learn about ICCS and IPIG recommendations for who should be tested for PNH.

Some common symptoms in a patient with PNH can be manifestations of devastating consequences from intravascular hemolysis. Learn how a full clinical assessment can uncover signs and symptoms associated with early mortality in your patient with PNH.

References: 1. Brodsky RA. Blood Rev. 2008;22:65-74. 2. Hillmen P, Lewis SM, Bessler M, et al. N Engl J Med. 1995;333:1253-1258. 3. Rachidi S, Musallam KM, Taher AT. Eur J Intern Med. 2010;21:260-267. 4. Rosse WF. In: Hoffman R, Benz EJ Jr, Shattil SJ, et al, eds. Hematology: Basic Principles and Practice. 3rd ed. New York, NY: Churchill Livingstone; 2000:331-342. 5. 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. 6. Rother RP, Bell L, Hillmen P, et al. JAMA. 2005;293:1653-1662. 7. Hillmen P, Elebute M, Kelly R, et al. Am J Hematol. 2010;85:553-559. 8. 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. 9. Kelly R, Richards S, Hillmen P, et al. Ther Clin Risk Manag. 2009;5:911-921. 10. Tsai, C-W, Wu V-C, Lin W-C, et al. Kidney Int. 2007;71:1187. 11. Hill A, Sapsford RJ, Scally A, et al. Br J Haematol. 2012, 158.409-414. 12. Hill A, Richards SJ, Hillmen P. Br J Haematol. 2007;137:181-192. 13. Hillmen P, Muus P, Dührsen U, et al. Blood. 2007;110:4123-4128. 14. Adams T, Fleischer D, Marino G, et al. Dig Dis Sci. 2002;47:58-64. 15. Meyers G, Weitz I, Lamy T, et al. Blood. 2007;110: Abstract 3683. 16. Hill A, Rother RP, Wang X, et al. Br J Haematol. 2010;149:414-425. 17. Parker C, Omine M, Richards S, et al; for International PNH Interest Group. Blood. 2005;106:3699-3709. 18. Nishimura J-I, Kanakura Y, Ware RE, et al. Medicine. 2004;83:193-207. 19. Dacie JV, Lewis SM. Ser Haematol. 1972;5:3-23.