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What is PNH?
PNH: Progressive. Destructive. Life Threatening.1-3
Paroxysmal nocturnal hemoglobinuria (PNH) is a progressive and destructive disease that can cause thrombosis, end-organ damage, and increased mortality.1-3
35% of Patients With PNH Die Within 5 Years of Diagnosis1
PNH is a rare disorder. The median age of patients when the disease presents is the early 30s, but PNH can occur in all age groups.2
PNH research has shown that at 5 years postdiagnosis, 35% of patients with PNH had died.1 The chart below shows the expected survival rate of patients with PNH compared with age- and gender-matched controls.
- Thrombosis is the leading cause of death
- Renal failure is also associated with mortality1-3
- In a patient population in which half the patients have <30% clone, 1 in 7 patients died within 5 years4
- Diagnosis is typically delayed from 1 to more than 10 years
Next: Pathophysiology of PNH
1. Hillmen P, Lewis SM, Bessler M, Luzzatto L, Dacie JV. N Engl J Med. 1995;333:1253-1258.
2. Socié G, Mary J-Y, de Gramont A, et al; for the French Society of Haematology. Lancet. 1996;348:573-577. 3. Nishimura J-I, Kanakura Y, Ware RE, et al. Medicine. 2004;83:193-207.
4. Peffault de Latour R, Mary JY, Salanoubat C, et al. Blood. 2008;112:3099-3106.
2. Socié G, Mary J-Y, de Gramont A, et al; for the French Society of Haematology. Lancet. 1996;348:573-577. 3. Nishimura J-I, Kanakura Y, Ware RE, et al. Medicine. 2004;83:193-207.
4. Peffault de Latour R, Mary JY, Salanoubat C, et al. Blood. 2008;112:3099-3106.
Dr Wendell Rosse explains how the pathophysiology of PNH can lead to thrombosis.