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Early diagnosis is essential for improved patient management and prognosis1,2
- Approximately 20%-35% of patients with PNH (receiving historical supportive care, including oral anticoagulation therapy and transfusions) die within 5-10 years of diagnosis3-6
- Chronic complement-mediated hemolysis is the underlying cause of progressive morbidities and premature mortality in PNH7,8
- 64% of patients with PNH have chronic kidney disease (CKD), which in advanced stages is associated with premature mortality9
- Nearly 50% of patients with PNH have evidence of pulmonary hypertension (PHT)10
- Establish a definitive diagnosis via high-sensitivity flow cytometry, a gold-standard diagnostic test for PNH7,11,12
- Hemolysis-induced complications increase the risk of premature mortality in patients with PNH3-5
- Monitoring is essential in patients with PNH who have elevated LDH and associated symptoms13
Patients with PNH showing signs and symptoms of hemolysis are at an increased risk for TEs and premature mortality13
Measuring LDH will provide a more complete clinical picture. LDH levels provide a means to monitor intravascular hemolysis in patients with PNH.14
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.11,15
All patients with PNH are at risk for thrombotic events (TEs) regardless of clone size13
- 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)13
- After the first TE occurs, patients with PNH have a greater risk of recurrent TEs and a 5- to 10-fold increase in mortality16
In PNH, fatigue and impaired quality of life (QoL) are independent of clone size17
Study description: Data from 524 patients in a global PNH registry from 78 clinical sites in 14 countries were analyzed to determine symptom presentation. Parameters examined included demographics, laboratory values, PNH clone size, medical history, symptoms, TE, and treatments.
- Even patients with clone size <10% experienced substantial impact on QoL17
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. Jang JH, et al. J Korean Med Sci. 2016;31(2):214-221. 2. Borowitz MJ, et al. Cytometry B Clin Cytom. 2010;78(4):211-230. 3. Hillmen P, et al. N Engl J Med. 1995;333(19):1253-1258. 4. Kelly RJ, et al. Blood. 2011;117(25):6786-6792. 5. Peffault de Latour R, et al. Blood. 2008;112(8):3099-3106. 6. Loschi M, et al. Am J Hematol. 2016;91(4):366-370. 7. Rachidi S, et al. Eur J Intern Med. 2010;21(4):260-267. 8. Hill A, et al. Br J Haematol. 2012;158(3):409-414. 9. Hillmen P, et al. Am J Hematol. 2010;85(8):553-559. 10. Hill A, et al. Br J Haematol. 2010;149(3):414-425.11. Parker C, et al. Blood. 2005;106(12):3699-3709. 12. Sharma VR. Clin Adv Hematol Oncol. 2013;11 Suppl 13(9):2-8. 13. Lee JW, et al. Int J Hematol. 2013;97(6):749-757. 14. Sahin F, et al. Am J Blood Res. 2016;6(2):19-27. 15. Parker CJ. Hematology Am Soc Hematol Educ Program. 2016;2016(1):208-216. 16. Socie G, et al. Lancet. 1996; 348(9027):573-577. 17. Urbano-Ispizua A, et al. The Hematol J. 2011: 422-422.