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Early diagnosis and intervention are critical
- 35% of patients with PNH die within 5 years of diagnosis2
- Chronic complement-mediated hemolysis is the underlying cause of progressive morbidities and premature mortality in PNH1,10
- 64% of patients with PNH have chronic kidney disease (CKD), which in advanced stages is associated with premature mortality4,9
- Nearly 50% of patients with PNH have evidence of pulmonary hypertension (PHT)8
- Due to the life-threatening and progressive nature of PNH, high-risk patients should be tested with the gold standard diagnostic test: high-sensitivity flow cytometry, performed on peripheral blood 1,3,7,11
- Hemolysis is a risk factor for TE and premature mortality12
- Rapid intervention and monitoring is essential in patients with PNH who have elevated LDH and associated symptoms5
LDH is the most important measure of intravascular hemolysis
Measuring LDH will provide a more complete clinical picture. LDH levels provide a means to monitor intravascular hemolysis in patients with PNH.6
It is important to establish a baseline LDH level and continue monitoring over time. Static or “snapshot”
measurements of LDH may not reflect the chronic and progressive course of the disease.7
Patients are at risk for TE regardless of clone size5
- Even patients with smaller clone sizes can experience thrombosis, and there was no evidence of any association between clone size category and risk of experiencing TE (P=0.843)5
In PNH, fatigue and impaired quality of life (QoL) are independent of clone size13
- Even patients with clone size <10% experienced substantial impact on QoL13
Support scientific collaboration in the PNH community by helping offer the international community greater insight into an uncommon disease with potentially devastating consequences. Enroll your patients in the PNH Registry today.
References: 1. Brodsky RA. Advances in the diagnosis and therapy of paroxysmal nocturnal hemoglobinuria. Blood Rev. 2008;22:65-74. 2. Hillmen P, Lewis SM, Bessler M, et al. Natural history of paroxysmal nocturnal hemoglobinuria. N Engl J Med. 1995;333:1253-1258. 3. 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. 4. Hillmen P, Elebute M, Kelly R, et al. Am J Hematol. 2010;85:553-559. 5. 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. 6. Urbano-Ispizua A, Schrezenmeier H, Brodsky R, et al. Haematolica. 2010: 95(S2):422. 7. Parker C, Omine, M, Richards S, et al; for International PNH Interest Group. Blood. 2005; 106:3699-3709. 8. Hill A, Rother RP, Wang X, et al. Br J Haematol. 2010;149:414-425. 9. 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. 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. Sharma VR. Paroxysmal nocturnal hemoglobinuria: pathogenesis, testing, and diagnosis. Clin Adv Hematol Oncol. 2013;11:1-11. 12. Kim JS, Lee JW, Yoon SS, et al. Association between elevated hemolysis at diagnosis and early mortality and risk of thrombosis in paroxysmal nocturnal hemoglobinuria (PNH) patients with cytopenia. Blood. 2010;116: Abstract 4241. 13. Urbano-Ispizua A, Schrezenmeier H, Brodsky R, et al. Evaluation of paroxysmal nocturnal hemoglobinuria disease burden in patients enrolled in the International PNH Registry. In: Abstracts of the 15th Congress of the European Hematology Association; June 10-13, 2010; Barcelona, Spain. Abstract 1022.