title: Leukotriene pathway in sickle cell disease: a potential target for directed therapy.
abstract: Sickle cell disease (SCD) is characterized by recurrent episodes of vaso-occlusion, resulting in tissue ischemia and end-organ damage. Inflammation is critical to the pathogenesis of vaso-occlusion and has been associated with SCD-related morbidity and mortality. Despite the impact of inflammation, no directed anti-inflammatory therapies for the treatment or prevention of vaso-occlusive events currently exist. Among individuals with SCD, asthma is a comorbid inflammatory condition that increases the risk of pain episodes, acute chest syndrome and death. Inflammation associated with asthma could augment the proinflammatory state of SCD, increasing episodes of vaso-occlusion. Leukotrienes are inflammatory mediators that play a prominent role in the pathogenesis of asthma and have been associated with SCD-related morbidity. Targeting inflammatory mediators, such as leukotrienes, is a promising approach for the development of novel therapies for the treatment of SCD. This review will examine the relationship between inflammation and vaso-occlusion, with particular focus on the leukotriene pathway.
authors.affiliation: Department of Internal Medicine, Washington University School of Medicine, St Louis, MO, USA.
authors.forename: Jessica
authors.initials: J
authors.lastname: Knight-Perry
authors.forename: Michael R
authors.initials: MR
authors.lastname: DeBaun
authors.forename: Robert C
authors.initials: RC
authors.lastname: Strunk
authors.forename: Joshua J
authors.initials: JJ
authors.lastname: Field
mesh_terms: D000755:Anemia, Sickle Cell / Q000188:drug therapy* / Q000378:metabolism* / Q000473:pathology; D000818:Animals; D006801:Humans; D015289:Leukotrienes / Q000378:metabolism*; D058990:Molecular Targeted Therapy
chemical_list: D015289:Leukotrienes