Priapism in patients with hemolytic disorders: a nationwide retrospective cohort study.

Priapism is a persistent, painful erection, which can lead to permanent penile damage and reduced quality of life. Patients with sickle cell disease have an increased risk of priapism which has been related to chronic hemolysis. This study investigates the prevalence of priapism in all major hereditary and acquired forms of hemolytic disorders. Patients with hemolytic disorders were identified in the nationwide Danish Hemolysis Cohort. Each patient was age-sex-matched with 50 comparisons from the general population without hemolysis. We identified the episodes of hospital-registered priapism events for both patients with hemolysis disorders and comparisons in the Danish National Patient Register between 1977 and 2016. We identified 4181 male patients with hemolytic disorders and 205,994 male comparisons, with 2,294,027 person-years of total observation time. Totally, 101 episodes of priapism occurred during follow-up period. Six episodes of priapism were recorded in three patients with a hemolytic disorder, all affected by sickle cell disease. Two of these patients had verified genotype HbSS. The incidence rate for first priapism in sickle cell disease was 432.8 per 100,000 person-years [95% CI: 139.6; 1341.8] versus 0.84 per 100,000 person-years [95% CI 0.54; 1.32] in comparisons. Using a large nationwide cohort, we found that only sickle cell disease is associated with priapism among patients with hemolytic disorders. The incidence rate of priapism in patients with sickle cell disease was lower than previously reported.

Early Mortality in Paroxysmal Nocturnal Hemoglobinuria.

OBJECTIVES:  The elevated mortality risk among patients with paroxysmal nocturnal hemoglobinuria (PNH) has been suggested to derive from a high risk of thromboembolism (TE); however, the risks of coexisting cardiovascular risk factors are not well described. We studied mortality associated with PNH taking comorbidity and treatment into account. METHODS: Patients with PNH (n=115) were identified in the 1977-2016 Danish National Patient Register (DNPR). For each patient with PNH, we identified 50 age- and sex-matched general population comparators. Using the Kaplan-Meier estimator and Cox regression, we compared the overall survival of patients with comparators. Cumulative incidences were used to analyze the effects of comorbidity and the causes of death. RESULTS: One-year survival among patients and comparators was 92.2% and 99.4%, and after 10 years, it was 68.4% and 85.8%, respectively. Early mortality was associated with older age, higher levels of comorbidity, and solid malignancies prior to PNH diagnosis. The leading causes of death were infections and associated hematological diseases. Patients with early mortality were less likely to have received treatment with eculizumab and/or warfarin. Cardiovascular risk factors were evenly distributed between patients and comparators at diagnosis. CONCLUSION: We conclude that early mortality in PNH is associated with older age, cardiovascular comorbidity, and hematological malignancies.