The epidemiology, evaluation and treatment of stroke in adults with sickle cell disease

Abstract

Stroke is a frequent and severe complication in adults with sickle cell disease. Ischemic stroke often causes physical and cognitive disability, while hemorrhagic stroke has a high mortality rate. As more children survive, the number of strokes in adults is increasing, yet stroke remains poorly understood. We review the epidemiology of ischemic and hemorrhagic stroke in adults with sickle cell disease and outline a practical approach to the evaluation of stroke including both sickle cell disease specific and general risk factors. We discuss the acute treatment and secondary prevention of stroke in this population based on the evidence in children with sickle cell disease and the general population, in addition to the limited studies in adults with sickle cell disease.

Keywords::

• epidemiology
• hydroxyurea
• intracranial hemorrhage
• ischemic stroke
• sickle cell disease
• subarachnoid hemorrhage
• transfusion

Financial & competing interests disclosure

JJ Strouse was supported by the NIH (grant number K23HL078819). The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

No writing assistance was utilized in the production of this manuscript.