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Updated: Mar 15, 2026

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
07:24

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy

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Sickle cell disease

J Strouse1

  • 1Division of Hematology, Department of Medicine and Division of Pediatric Hematology/Oncology, Department of Pediatrics, Duke University, Durham, NC, USA.

Handbook of Clinical Neurology
|September 18, 2016
PubMed

Insights

Sickle cell disease (SCD) can cause serious central nervous system complications like stroke and cognitive impairment. Early detection and management of SCD complications are crucial for improving patient outcomes.

Area of Science:

  • Hematology
  • Neurology
  • Genetics

Background:

  • Sickle cell disease (SCD) is a common inherited blood disorder caused by HBB gene mutation.
  • It affects millions globally, with significant childhood incidence.
  • Central nervous system (CNS) injury, including stroke and cognitive impairment, is a frequent and debilitating complication.

Purpose of the Study:

  • To highlight the prevalence and impact of CNS complications in sickle cell disease.
  • To emphasize the link between silent cerebral infarcts (SCI) and cognitive deficits.
  • To underscore the cognitive impact of SCD, even in mild cases.

Main Methods:

  • Review of existing literature and studies on SCD complications.
  • Analysis of data on stroke incidence in children with sickle cell anemia (HbSS).
  • Assessment of cognitive function in children with and without SCI, and in adults with SCD.

Main Results:

  • 11% of children with HbSS experienced stroke before age 18 prior to screening implementation.
  • Silent cerebral infarcts (SCI) affect 27% of children with HbSS by age 5.
  • Children with SCI exhibit greater cognitive impairment; adults with mild SCD also show cognitive dysfunction.

Conclusions:

  • CNS complications, particularly stroke and SCI, are highly prevalent and impactful in SCD.
  • SCI is a significant predictor of cognitive impairment in children with SCD.
  • Cognitive dysfunction is a pervasive issue in SCD, affecting individuals across disease severity spectrums.

Keywords:
cognitive impairmenthemorrhagic strokeischemic strokesickle cell anemiasickle cell diseasesilent cerebral infarct

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