Managing the Cerebrovascular Complications of Sickle Cell Disease: Current Perspectives

Abstract

Jennifer Light,* Maria Boucher,* Jacquelyn Baskin-Miller,* Mike Winstead* Pediatric Hematology-Oncology, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA*These authors contributed equally to this workCorrespondence: Mike Winstead, Division of Pediatric Hematology-Oncology, University of North Carolina at Chapel Hill, 101 Manning Drive, Chapel Hill, NC, USA, Tel [phone], Fax [phone], Email [email protected]: The importance of protecting brain function for people with sickle cell disease (SCD) cannot be overstated. SCD is associated with multiple cerebrovascular complications that threaten neurocognitive function and life. Without screening and preventive management, 11% of children at 24% of adults with SCD have ischemic or hemorrhagic strokes. Stroke screening in children with SCD is well-established using transcranial Doppler ultrasound (TCD). TCD velocities above 200 cm/s significantly increase the risk of stroke, which can be prevented using chronic red blood cell (RBC) transfusion. RBC transfusion is also the cornerstone of acute stroke management and secondary stroke prevention. Chronic transfusion requires long-term management of complications like iron overload. Hydroxyurea can replace chronic transfusions for primary stroke prevention in a select group of patients or in populations where chronic transfusions are not feasible. Silent cerebral infarction (SCI) is even more common than stroke, affecting 39% of children and more than 50% of adults with SCD; management of SCI is individualized and includes careful neurocognitive evaluation. Hematopoietic stem cell transplant prevents cerebrovascular complications, despite the short- and long-term risks. Newer disease-modifying agents like voxelotor and crizanlizumab, as well as gene therapy, may treat cerebrovascular complications, but these approaches are investigational.Keywords: stroke, silent cerebral infarction, moyamoya syndrome, transfusion, stem cell transplantation

Keywords

• stroke
• silent cerebral infarction
• moyamoya syndrome
• transfusion
• stem cell transplantation