Early diagnosis is essential for improved patient management and prognosis. Learn about ICCS and other expert recommendations for who should be tested for PNH.
Chronic, complement-mediated hemolysis is the underlying cause of progressive morbidities and premature mortality in PNH1
- Even in the absence of symptoms, hemolysis is ongoing2
- The consequences of hemolysis can be unpredictable, sudden, and potentially fatal1,3
- Hemolysis-induced nitric oxide depletion results in thrombosis, abdominal pain, pulmonary hypertension, dysphagia, and erectile dysfunction4,5
- All patients with PNH are at risk for thrombotic events (TEs) regardless of clone size6
Rapid recognition of the signs and symptoms and early diagnosis of PNH are critical for disease management15,16
A diagnosis of PNH is typically delayed from 1 to more than 10 years.17 The name of the disease can be misleading, as nearly three-fourths of patients with PNH do not present with hemoglobinuria.18 The wide range of symptoms caused by PNH is shown in the figure at left.
A clinical assessment is crucial to determine the risk for morbidities and premature mortality in your patient with PNH10,14,18
aMale patients only (n=410).11
Some common symptoms in a patient with PNH can be manifestations of devastating consequences from chronic complement-mediated hemolysis. Learn how a full clinical assessment can uncover signs and symptoms associated with premature mortality in your patient with PNH.
References: 1. Sahin F, et al. Am J Blood Res. 2015;5(1):1-9. 2. Sahin F, et al. Am J Blood Res. 2015;5(1):30-33. 3. Brodsky RA. In: Hollman R, et al, eds. Hematology: Basic Principles and Practices. 4th ed. Philadelphia, PA: Elsevier Churchill Livingstone;2005:419-427. 4. Hillmen P, et al. Br J Haematol. 2013;162(1):62-73. 5. Rother RP, et al. JAMA. 2005;293(13):1653-1662. 6. Lee JW, et al. Int J Hematol. 2013;97(6):749-757. 7. Brodsky RA, et al. Blood. 2008;111(4):1840-1847. 8. Brodsky RA. In: Hoffman R, et al, eds. Hematology: Basic Principles and Practice. Philadelphia, PA: Saunders/Elsevier; 2013:373-382. 9. Hill A, et al. Blood. 2013;121(25):4985-4996. 10. Weitz I, et al. Intern Med J. 2013;43(3):298-307. 11. Schrezenmeier H, et al. Haematologica. 2014;99(5):922-929 and Supplemental Material. 12. Nishimura J-I, et al. Medicine. 2004;83(3):193-207. 13. Sharma VR. Clin Adv Hematol Oncol. 2013;11 Suppl 13(9):2-8. 14. Hill A, et al. Br J Haematol. 2010;149(3):414-425. 15. Borowitz MJ, et al. Cytometry B Clin Cytom. 2010;78(4):211-230.16. Jang JH, et al. J Korean Med Sci. 2016;31(2):214-221.17. Dacie JV, et al. Ser Haematol. 1972;5(3):3-23.18. Parker C, et al. Blood. 2005;106(12):3699-3709.