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Vaso-occlusion in sickle cell disease: pathophysiology and novel targeted therapies

2013; Elsevier BV; Volume: 122; Issue: 24 Linguagem: Inglês

10.1182/blood-2013-05-498311

1528-0020

Deepa Manwani, Paul S. Frenette,

Iron Metabolism and Disorders

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.