PNH Morbidity and Mortality
Chronic hemolysis is central to the morbidities and mortality of PNH
Hemolysis is chronic in PNH, and is central to its characteristic signs and symptoms, illustrated in the diagram below.
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Thrombosis
- Is the leading cause of death in PNH, occurring in approximately 40% of patients
- An estimated 50% of all PNH deaths may be attributed to thrombosis
- Thrombosis has been reported to increase the risk of death 10-fold in patients with PNH
Anemia
- Presents in a majority of PNH patients and is caused by chronic hemolysis and/or bone marrow failure
- Patients may become transfusion-dependent to maintain adequate levels of hemoglobin
Smooth muscle dystonias
- Include dysphagia, abdominal pain, erectile dysfunction in men and pulmonary and systemic hypertension
- Are hypothesized to be related to nitric oxide depletion during chronic hemolysis
Impaired quality of life
- Quality of life is frequently impaired in patients with PNH, given the range of signs, symptoms and co-morbidities that often exist in these patients
- Symptoms can include disabling fatigue, difficulty performing daily activities, pain and dyspnea
Next: What Are the Symptoms of PNH?