Nearly 50% of PNH Patients Suffer from Pulmonary Hypertension (PHT)1
PHT can be linked to hemolysis and resulting nitric oxide (NO) depletion.
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Hemolysis levels and NO consumption were shown to be much greater in patients with PNH than in patients with other hemolytic diseases (6- to 10-fold, respectively)1
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Evidence of PHT in PNH patients has been observed using 2 key measures for PHT1
- Doppler echocardiography
- NT-proBNP level (≥160 pg/mL)
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PNH patients with PHT present with cardiac dysfunction1
Symptoms of PHT commonly found in patients with PNH.2
- 66% of patients reported shortness of breath2
- 72% of patients qualified as moderate to severe2
- 88% reported distress due to dyspnea2
Other PNH symptoms that may be associated with nitric oxide deficiency include abdominal pain, dysphagia, and erectile dysfunction.
References: 1. Hill A, Rother RP, Wang X, et al. Eculizumab reduces pulmonary hypertension through inhibition of hemolysis-associated nitric oxide consumption in patients with paroxysmal nocturnal hemoglobinuria [ASH abstract]. Blood. 2008;112: Abstract 486.
2. Meyers G, Weitz I, Lamy T, et al. Disease-related symptoms reported across a broad population of patients with paroxysmal nocturnal hemoglobinuria [ASH abstract]. Blood. 2007;110: Abstract 3683.