Transcranial Doppler Velocities among Sickle Cell Disease Patients in Steady State

Abstract

Transcranial Doppler (TCD) screening is an established tool to identify children with sickle cell disease at high risk of stroke. Our objective was to study TCD velocities among sickle cell disease patients while in a steady state. This cross-sectional study included 78 steady state sickle cell disease patients [31 Hb SS (β^S/β^S) (sickle cell anemia), 47 Hb S/β-thalassemia (HBB: c.20A>T/β-thal)], attending the Pediatric Hematology Clinic at Cairo University Children’s Hospital, Cairo, Egypt. All patients underwent TCD velocity assessment as per the Stroke Prevention Trial in Sickle Cell Anemia (STOP) protocol. In our cohort, TCD velocities were comparable among Hb S/β-thal vs. SS patients. Hemolysis indicators correlated significantly to TCD velocities in Hb S/β-thal patients; positive correlation was found between total bilirubin level and right middle cerebral artery (MCA) and right distal internal carotid artery (dICA) TCD velocities (r = 0.428, p = 0.00, r = 0.360, p = 0.01), respectively as well as between reticulocyte count and right MCA, right dICA and right anterior cerebral artery (ACA) TCD velocities (r = 0.424, p = 0.01), (r = 0.40, p = 0.00), (r = 0.303, p = 0.04), respectively. On the other hand, statistically significant negative correlations were found between hemoglobin (Hb) level and right ACA, right dICA TCD velocities (r = −0.290, p = 0.05), (r = −0.324, p = 0.03). Although Hb F is considered an ameliorating factor for disease severity; hemolysis stands as an indicator of risk for TCD velocity elevation, and in turn, risk for stroke among sickle cell disease patients.

Keywords:

• Hemolysis
• sickle cell anemia
• sickle cell disease
• steady state
• transcranial Doppler (TCD)

Acknowledgements

The authors would like to thank all staff members of the Pediatric Hematology Clinic at Cairo University Children’s Hospital, Cairo, Egypt for the detailed and accurate documentation of patients’ follow-up in the medical records.

Disclosure statement

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of this article.