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.

Moderate fixed‐dose hydroxyurea for primary prevention of strokes in Nigerian children with sickle cell disease: Final results of the SPIN trial

“…An early (3 months) and sustained (2 years) decrease in the initial abnormal TCD measurements was unexpected, but an important finding in our clinical trial (Figure 1). The results from this trial bolster pre‐existing pooled data from 10 studies demonstrating that hydroxyurea therapy significantly lowers TCD velocity 6 …”

“…In summary, the results of our NIH/NINDS‐funded feasibility SPIN Trial provide additional evidence supporting the American Society of Hematology recommendation that for children with SCA and with abnormal TCD measurements living in a low‐ and middle‐income settings, initial treatment with moderate fixed‐dose hydroxyurea (~20 mg/kg/d) is comparable to initial treatment with regular blood transfusion in the STOP trial (0.9 per 100 person‐years with a total of 110 patient‐years) and superior to no treatment 6 . In addition, monthly CBC assessments for myelosuppression associated with moderate fixed‐dose hydroxyurea have limited clinical utility in low‐ and middle‐income settings.…”

A user guide to the American Society of Hematology clinical practice guidelines

“…Since November 2018, Blood Advances has published American Society of Hematology (ASH) clinical practice guidelines on venous thromboembolism (VTE), [1][2][3][4][5][6][7][8] immune thrombocytopenia, 9 and sickle cell disease. [10][11][12] More ASH guidelines on these and other topics are forthcoming. In this article, we describe why ASH guidelines are trustworthy, and how patients, clinicians, policymakers, researchers, and others may use them.…”

Practice patterns for neuroimaging and transfusion therapy for management of neurologic complications in sickle cell anemia: DISPLACE consortium

“…Table 1 provides a summary of the 2014 NHLBI guidelines on neuroimaging and CRCT 3 . The 2020 ASH guidelines pertaining to these practices are described in Table 2 5,6 . Regarding neuroimaging, the 2014 NHLBI guidelines did not provide a firm recommendation for or against MRI use for screening asymptomatic children with SCA for stroke.…”