Clinical Practice Guidelines on CNS Complications in Sickle Cell Disease (ASH, 2020)

American Society of Hematology

This is a quick summary of the guidelines without analysis or commentary. For more information, go directly to the guidelines by clicking the link in the reference.

May 28, 2020

In April 2020, the American Society of Hematology published guidelines on the prevention, diagnosis, and treatment of central nervous system (CNS) complications in sickle cell disease.[1]

Primary stroke prevention in children with sickle cell disease (SCD) occupying low-middle– and high-income settings

Annual transcranial Doppler (TCD) screening is recommended for children aged 2-16 years with hemoglobin SS (HbSS) or HbSβ0 thalassemia.

Regular blood transfusions for a minimum of 1 year are recommended for children aged 2-16 years with HbSS or HbSβ0 thalassemia who have abnormal TCD velocities and live in a high-income setting. This refers to a setting that permits regular blood transfusion therapy, typically every 3-4 weeks, to maintain the maximum HbS level below 30% and the hemoglobin level above 9.0 g/dL, to reduce stroke risk.

Management of suspected or confirmed ischemic stroke or transient ischemic attack (TIA)

Prompt blood transfusion is recommended for children or adults with SCD and acute neurologic deficits, including transient ischemic attack (TIA). The transfusion should not be delayed beyond 2 hours of acute neurologic symptom presentation. Individual patient factors and local transfusion resources determine the type of transfusion provided (simple, modified exchange, or apheresis).

Secondary prevention of ischemic strokes in children and adults with HbSS or HbSβ0 thalassemia

Assessment for revascularization surgery as an adjunct to regular blood transfusion is suggested for adults and children with SCD, moyamoya syndrome, and a history of stroke or TIA.

Rehabilitation for children and adults with cognitive impairments

The following are recommended for children with SCD and abnormal developmental or cognitive status screens:

  • Developmental, cognitive, and medical assessment to diagnose any related disorders and to recognize any modifiable risk factors for developmental delays or cognitive impairments

  • Delivery of appropriate interventions by following the cognitive domain–specific, evidence-based guidelines for these disorders

The following are recommended for adults with SCD and abnormal cognitive status screens:

  • Cognitive and medical assessment to diagnose any related disorders and to recognize any modifiable risk factors for cognitive impairments

  • Delivery of appropriate interventions by following the cognitive domain–specific, evidence-based guidelines for these disorders

Screening for silent cerebral infarcts in children and adults with HbSS or HbSβ0 thalassemia

At least a one-time magnetic resonance imaging (MRI) screening, without sedation, is recommended to detect silent cerebral infarcts in early school-aged children. This recommendation is made in view of the fact that silent cerebral infarcts are highly prevalent in children with HbSS or HbSβ0 thalassemia (1 in 3) and are associated with cognitive impairment, poor school performance, and future cerebral infarcts.

For more information, please go to Sickle Cell Anemia.

For more Clinical Practice Guidelines, please go to Guidelines.

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