Cerebral vasculopathy in children with sickle cell anemia

Ross M. Fasano, Emily R. Meier, Monica L. Hulbert

Research output: Contribution to journalReview articlepeer-review

26 Scopus citations

Abstract

Sickle cell anemia (SCA)-associated cerebral vasculopathy and moyamoya is a unique entity reflecting the abnormal interactions between sickled red blood cells (RBCs) and the cerebral arterial endothelium. Endothelial injury, coagulation activation, and the inflammatory response generated by sickled RBCs are implicated in the development of cerebral vasculopathy, but the pathophysiology remains incompletely understood. SCA-specific screening and treatment guidelines have successfully reduced the incidence of overt strokes in this high-risk population. However, despite aggressive hematological management, many children with cerebral vasculopathy due to SCA have progressive vasculopathy and recurrent strokes; therefore, more effective therapies, such as revascularization surgery and curative hematopoietic stem cell transplant, are urgently needed.

Original languageEnglish
Pages (from-to)17-25
Number of pages9
JournalBlood Cells, Molecules, and Diseases
Volume54
Issue number1
DOIs
StatePublished - Jan 1 2015

Keywords

  • Cerebral vasculopathy
  • Moyamoya
  • Sickle cell anemia
  • Stroke

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