ASH News Daily 2013 - Day 2 - (Page C-2)
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PNH
35
%
of patients with PNH* die within 5 years of diagnosis despite
best historical care5
* Chronic, complement-mediated intravascular hemolysis is the underlying cause of progressive
morbidities and mortality in PNH6
* 40% to 67% of deaths are due to venous or arterial thrombosis7
* 64% of patients with PNH have chronic kidney disease (CKD), which is associated with early mortality8
* Nearly 50% of patients with PNH have evidence of pulmonary hypertension9
* Routine testing for PNH in high-risk populations is warranted1,10
*PNH = paroxysmal nocturnal hemoglobinuria.
"We can safely say that PNH is the most vicious acquired thrombophilic state known in medicine."11
- Hill, Blood 2013
References: 1. Borowitz MJ, Craig FE, DiGiuseppe JA, et al; for Clinical Cytometry Society. Cytometry Part B. 2010;78B:211-230. 2. Richards SJ, Barnett D. Clin Lab Med. 2007;27:577-590. 3. Ariceta G, Besbas N,
Johnson S, et al; for the European Paediatric Study Group for HUS. Pediatr Nephrol. 2009;24:687-696. 4. Caprioli J, Noris M, Brioschi S, et al; for the International Registry of Recurrent and Familial HUS/TTP. Blood.
2006;108:1267-1279. 5. Hillmen P, Lewis SM, Bessler M, et al. N Engl J Med. 1995;333:1253-1258. 6. Brodsky RA. Blood Rev. 2008;22:65-74. 7. Hillmen P, Muus P, Dührsen U, et al. Blood. 2007;110:4123-4128. 8. Hillmen P,
Elebute M, Kelly R, et al. Am J Hematol. 2010;85:553-559. 9. Hill A, Rother RP, Wang X, et al. Br J Haematol. 2010;149:414-425. 10. Data on file. Alexion Pharmaceuticals, Inc.; 2013. 11. Hill A, Kelly R, Hillmen P. Blood.
2013;121:4985-4996. 12. Noris M, Caprioli J, Bresin E, et al. Clin J Am Soc Nephrol. 2010;5:1844-1859. 13. Langman CB. Haematologica. 2012;97(suppl 1): Abstract 0490. 14. Loirat C, Noris M, Frémeaux-Bacchi V.
Pediatr Nephrol. 2008;23:1957-1972. 15. Sallée M, Daniel L, Piercecchi M-D, et al. Nephrol Dial Transplant. 2010;25:2028-2032. 16. Neuhaus TJ, Calonder S, Leumann EP. Arch Dis Child. 1997;76:518-521. 17. Remuzzi G,
Ruggenenti P, Colledan M, et al. Am J Transplant. 2005;5:1146-1150. 18. Mache CJ, Acham-Roschitz B, Frémeaux-Bacchi V, et al. Clin J Am Soc Nephrol. 2009;4:1312-1316. 19. Vergouwen MD, Adriani KS, Roos YB, et al.
AJNR Am J Neuroradiol. 2008;29:e34. 20. Malina M, Gulati A, Majid MA, et al. Pediatr Nephrol. 2011;26:1678. 21. Larakeb A, Leroy S, Frémeaux-Bacchi V, et al. Pediatr Nephrol. 2007;22:1967-1970. 22. Loirat C, Macher M-A,
Elmaleh-Berges M, et al. Nephrol Dial Transplant. 2010;25:3421-3425. 23. Loirat C, Garnier A, Sellier-Leclerc A-L, et al. Semin Thromb Hemost. 2010;36:673-681.
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Table of Contents for the Digital Edition of ASH News Daily 2013 - Day 2
Table of Contents
ASH News Daily 2013 - Day 2
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