Pulmonary embolisms (PEs) in hospitalized children with sickle cell disease (SCD) might occur in 0.53% of the examined cases as well as those patients with higher disease severity, according to a new research article published in Pediatric Hematology and Oncology.

For the purpose of this study, the researchers reviewed the Pediatric Health Information System to identify patients with SCD aged 0 to 21 years who were admitted to a database-included hospital between January 2010 and June 2021.

The study authors examined numerous diagnostic codes and imaging, procedure, and pharmaceutical billing codes to gain insight into various PE cases, as well as potential clinical, demographic, and utilization risk factors. They also performed logistic regression analyses to evaluate the associations between risk factors and PEs.

According to the results, 22,631 SCD patients with a median age of 10.8 years were identified, and 120 (0.53%) of them, with a median age of 17.4 years, experienced PEs. Those affected by PEs utilized more healthcare resources than SCD patients without PEs, as evidenced by longer hospitalizations and more frequent ICU admissions.

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“Patients with PE demonstrated higher healthcare utilization characteristics indicating significant disease burden associated with PE. The implications are not only limited to increased cost expenditure but could also suggest that children with PE are at risk of other long term complications such as pulmonary hypertension and right ventricular dysfunction to which they [are] already predisposed due to underlying disease state,” Bala and colleagues noted.

In addition, the multivariable analysis revealed older age, prior history of central venous line, acute chest syndrome, and apheresis to be risk factors significantly associated with PEs. Mortality rates did not differ significantly between those with and without PEs.

“Clinicians should include PE in their differential diagnosis while evaluating children with SCD, especially older children or adolescents with a more severe clinical disease state (eg, presence of [central venous line], prior [acute chest syndrome], and prior apheresis) who present with signs and symptoms raising the concern for PE,” the study authors added.

Hypercoagulopathy in SCD is linked with chronic inflammation, platelet activation, endothelial dysfunction, and disorders of fibrinolysis. Although there is extensive research on the incidence and risk factors associated with PEs in adults with SCD, studies examining the rates of PEs in children with SCD are lacking.


Bala N, Stanek J, Rodriguez V, Villella A. Prevalence and risk factors for pulmonary embolism in pediatric sickle cell disease: a national administrative database study. Pediatr Hematol Oncol. Published online January 16, 2023. doi:10.1080/08880018.2023.2166634