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What neurologists need to understand outside their own speciality
Cerebrovascular disease in sickle cell disease
  1. Vafa Alakbarzade1,
  2. Chinedu Maduakor1,
  3. Usman Khan1,
  4. Nader Khandanpour2,
  5. Elizabeth Rhodes3,
  6. Anthony C Pereira1
  1. 1 Department of Neurology, St George's University Hospitals NHS Foundation Trust, London, UK
  2. 2 Department of Neuroradiology, St George's University Hospitals NHS Foundation Trust, London, UK
  3. 3 Department of Haematology, St George's University Hospitals NHS Foundation Trust, London, UK
  1. Correspondence to Dr Vafa Alakbarzade, Department of Neurology, St George's University Hospitals NHS Foundation Trust, London SW17 0QT, UK; vafa.alakbarzade.10{at}ucl.ac.uk

Abstract

Sickle cell disease (SCD) is the most common type of hereditary anaemia and genetic disorder worldwide. Cerebrovascular disease is one of its most devastating complications, with consequent increased morbidity and mortality. Current guidelines suggest that children and adults with SCD who develop acute ischaemic stroke should be transfused without delay. Those with acute ischaemic stroke aged over 18 years who present within 4.5 hours of symptom onset should be considered for intravenous thrombolysis; older patients with conventional vascular risk factors are the most likely to benefit. Endovascular thrombectomy should be considered carefully in adults with SCD as there are few data to guide how the prevalence of cerebral vasculopathy may confound the expected benefits or risks of intervention. We present a practical approach to cerebrovascular disease in sickle cell patients based on the available evidence and our experience.

  • stroke
  • haematology

Data availability statement

Data are available upon reasonable request.

https://doi.org/10.1136/pn-2022-003440

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    Data availability statement

    Data are available upon reasonable request.

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    Footnotes

    • Contributors ACP and ER conceived the study. VA and CM collected data. ACP, VA, UK and ER revised the manuscript for content, including medical writing for content. VA drafted the initial manuscript. NK revised the neuroimaging. All authors gave final approval for publication.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Provenance and peer review Commissioned; externally peer reviewed by David Werring, London, UK.