Which adults with sickle cell disease need an evaluation for pulmonary embolism?

Abstract

Individuals living with sickle cell disease (SCD) are at an increased risk of venous thrombo-embolism (VTE) including pulmonary embolisms (PEs). There is a high mortality associated with PE in individuals with SCD. It can be difficult to diagnose PE since presenting symptoms of PE often mimic those of other forms of vaso-occlusive crisis in SCD. Currently, there are no validated models for predicting PEs in patients with sickle cell disease, which often leads to frequent CT scans and exposure to harmful radiation and intravenous contrast. The aim of this study was to evaluate different host variables and potential clinical biomarkers of patients with SCD including those used in the Wells score to assess predictability for PE in order to create a more accurate diagnostic algorithm to predict PE. A retrospective chart review was performed on 349 patients with SCD who underwent testing for a PE with a CT scan of the chest. Forward and backward stepwise model selection was performed to obtain a parsimonious model of the predictors of PEs. The incidence of PE in this population was 9·7%. Of the factors evaluated for this study, the Wells score was the only one with clinical significance. A Wells score greater than 4 had a sensitivity and specificity of 72·5% and 70·1%, respectively, and a score greater than 6 had a sensitivity and specificity of 50% and 87%, respectively. The Wells score is an acceptable clinical tool which may prove useful in individuals with SCD to predict who is most likely to have a PE and therefore should undergo a CT scan. A prospective study is needed to further confirm these findings.