Abstract
The threshold velocity ≥200 cm/s at transcranial Doppler (TCD) evaluation is a useful cut-off for preventing the stroke (STOP trial) in pediatric patients with sickle cell disease (SCD), term including different types of sickle genotypes. Scanty data are available for adult SCD patients. We compared intracranial blood flow velocities between adult SCD patients and controls using transcranial color Doppler (TCCD), measuring the peak of systolic velocity (PSV) with the insonation angle correction and the pulsatility index (PI), an indicator of endothelial elasticity. Fifty-three adult SCD patients (aged >18 years) were enrolled (15 sickle cell anemia, 26 sickle cell thalassemia, and 12 HbS/HbC). None of the patients presented neurological signs. PSVs in middle cerebral artery (MCA) were higher in SCD patients than in controls (p = 0.001). In sickle cell anemia patients, PSVs were higher when compared to HbS/βThal (p < 0.0060) and HbS/HbC patients (p < 0.0139). PI was within the lower range of normality in SCD patients compared to controls. Moreover, MCA-PSV was higher with lower Hb levels and higher HbS%; PI did not change with variation of Hb levels and HbS%.
PSV and PI in SCD adult patients could be a relevant index to indicate the abnormal cerebral blood flow and to detect the sickle endothelial damage, in order to prevent cerebrovascular accidents.
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Acknowledgments
We thank all the patients for participation in this study. GG and FMC coordinated the study design and contributed to data preparation and analysis; GG and FMC wrote the paper; FA and FMC did transcranial color Doppler; PR and AM recruited the patients; PR, IG, and EDP collected data; IC made statistical analysis; MDC contributed to review and final approval for submission. All authors read and agreed to the final version of the manuscript. This work was supported by Ministry of Health, Italy [RC-2016].
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The study was approved by the Ethical Committee of our Institution and the procedures were performed in accordance with the Declaration of Helsinki. All subjects received verbal and written explanation of the aims and procedure of the study and written informed consent was obtained.
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Graziadei, G., Casoni, F.M., Annoni, F. et al. Transcranial color Doppler in stroke-free adult patients with sickle cell disease. Ann Hematol 96, 1547–1555 (2017). https://doi.org/10.1007/s00277-017-3071-1
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DOI: https://doi.org/10.1007/s00277-017-3071-1