Blood advances 2020 Apr 28
American Society of Hematology 2020 guidelines for sickle cell disease: prevention, diagnosis, and treatment of cerebrovascular disease in children and adults.
Abstract
Background
Central nervous system (CNS) complications are among the most common, devastating sequelae of sickle cell disease (SCD) occurring throughout the lifespan.
Objective
These evidence-based guidelines of the American Society of Hematology are intended to support the SCD community in decisions about prevention, diagnosis, and treatment of the most common neurological morbidities in SCD.
Methods
The Mayo Evidence-Based Practice Research Program supported the guideline development process, including updating or performing systematic evidence reviews. The panel used the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach, including GRADE evidence-to-decision frameworks, to assess evidence and make recommendations.
Results
The panel placed a higher value on maintaining cognitive function than on being alive with significantly less than baseline cognitive function. The panel developed 19 recommendations with evidence-based strategies to prevent, diagnose, and treat CNS complications of SCD in low-middle- and high-income settings.
Conclusions
Three of 19 recommendations immediately impact clinical care. These recommendations include: use of transcranial Doppler ultrasound screening and hydroxyurea for primary stroke prevention in children with hemoglobin SS (HbSS) and hemoglobin Sβ0 (HbSβ0) thalassemia living in low-middle-income settings; surveillance for developmental delay, cognitive impairments, and neurodevelopmental disorders in children; and use of magnetic resonance imaging of the brain without sedation to detect silent cerebral infarcts at least once in early-school-age children and once in adults with HbSS or HbSβ0 thalassemia. Individuals with SCD, their family members, and clinicians should become aware of and implement these recommendations to reduce the burden of CNS complications in children and adults with SCD.
Related Questions
How long would you continue chronic transfusion therapy for adult sickle cell patients who have had a stroke?
There is no high-level evidence to inform the decision of how long to continue chronic transfusion in this scenario. Decisions on whether to continue indefinitely are affected by multiple factors like the presence of RBC alloimmunization, whether the patient was on hydroxyurea at MTD or not at the t...
How would you approach secondary stroke prevention in an adult with Hemoglobin SC disease?
Stroke is less common in HbSC disease than it is in HbS homozygotes (Ohene-Frempong et al., PMID 9414296). Thus, there are no studies focused on primary or secondary stroke prevention in HbSC disease. Recent guidelines for stroke management were “silent” on stroke in HbSC disease (DeBaun et al., PMI...