Leukotriene pathway in sickle cell disease: a potential target for directed therapy

Expert Rev Hematol. 2009 Feb;2(1):57-68. doi: 10.1586/17474086.2.1.57.

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.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Anemia, Sickle Cell / drug therapy*
  • Anemia, Sickle Cell / metabolism*
  • Anemia, Sickle Cell / pathology
  • Animals
  • Humans
  • Leukotrienes / metabolism*
  • Molecular Targeted Therapy

Substances

  • Leukotrienes