Early mortality in paroxysmal nocturnal hemoglobinuria (PNH) is associated with older age, cardiovascular comorbidity, and hematologic malignancies, according to findings published in Cureus. Henrik Frederiksen, MD, PhD, and colleagues examined mortality associated with PNH, accounting for comorbidities and treatment, among 115 patients with PNH (median age at diagnosis, 48.2) compared with matched individuals from the general population. The presence of cardiovascular risk factors was equally distributed between groups. Survival at 1 year was 92.2% for patients with PNH versus 99.4% for matched individuals; survival at 10 years was 68.4% and 85.8%, respectively. Infections and associated hematologic conditions were the primary causes of death, and early mortality was associated with older age, greater comorbidity, and the development of solid malignancies before a PNH diagnosis. Among patients with PNH, 18 died within 2 years of diagnosis. “The rarity of the disease calls for international collaboration to investigate rare events and complications associated with PNH, particularly when subgroups such as elderly patients are considered,” Dr. Frederiksen and colleagues wrote.
