Vaso-occlusion in sickle cell disease: pathophysiology and novel targeted therapies.
Recurrent and unpredictable episodes of vaso-occlusion are the hallmark of sickle cell disease. Symptomatic management and prevention of these events using the fetal hemoglobin-reactivating agent hydroxyurea are currently the mainstay of treatment. Discoveries over the past 2 decades have highlighted the important contributions of various cellular and soluble participants in the vaso-occlusive cascade. The role of these elements and the opportunities for therapeutic intervention are summarized in this review.
Departments of Pediatrics.
Deepa
D
Manwani
Paul S
PS
Frenette
D000755:Anemia, Sickle Cell / Q000188:drug therapy* / Q000503:physiopathology*; D000986:Antisickling Agents / Q000627:therapeutic use; D002448:Cell Adhesion / Q000502:physiology; D004730:Endothelium, Vascular / Q000503:physiopathology; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use*; D008954:Models, Biological; D009504:Neutrophils / Q000502:physiology; D014652:Vascular Diseases / Q000503:physiopathology*
D000986:Antisickling Agents; D006918:Hydroxyurea