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Vaso-occlusion in sickle cell disease: pathophysiology and novel targeted therapies
Deepa Manwani1, Paul S Frenette
1Departments of 1Pediatrics.
Abstract:
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.
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