title: Pathology of sickle cell disease.
abstract: Sickle cell disease (SCD) is a hereditary chronic hemolytic anemia with numerous clinical consequences. Intravascular sickling of red blood cells leads to multiorgan dysfunction. Although the pathophysiology of SCD has been well studied, there remains a lack of effective treatment. Refinements in overall care have improved quality of life; however, premature death is still not uncommon. SCD usually presents in childhood and is common in areas where malaria is (or was) common. The association with malaria is apparently of benefit to the individual because these individuals tend to contract a milder form of the disease. This review highlights the spectrum of pathology seen in people with SCD, with an emphasis on the pathogenesis of sudden death.
authors.affiliation: Department of Pathology and Laboratory Medicine, Toronto General Hospital/University Health Network, Toronto, Ontario, Canada.
authors.forename: Janet I
authors.initials: JI
authors.lastname: Malowany
authors.forename: Jagdish
authors.initials: J
authors.lastname: Butany
mesh_terms: D000755:Anemia, Sickle Cell / Q000188:drug therapy / Q000473:pathology* / Q000503:physiopathology; D000986:Antisickling Agents / Q000627:therapeutic use; D003645:Death, Sudden / Q000473:pathology; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use; D061213:Mortality, Premature; D009102:Multiple Organ Failure / Q000473:pathology; D011788:Quality of Life
chemical_list: D000986:Antisickling Agents; D006918:Hydroxyurea