Vasculopathy in sickle cell disease: Biology, pathophysiology, genetics, translational medicine, and new research directions.

ABSTRACT: Sickle cell disease has been very well characterized as a single amino acid molecular disorder of hemoglobin leading to its pathological polymerization, with resulting red cell rigidity that causes poor microvascular blood flow, with consequent tissue ischemia and infarction. More recently, an independent spectrum of pathophysiology of blood vessel function has been demonstrated, involving abnormal vascular tone and activated, adhesive endothelium. These vasculopathic abnormalities are attributable to pathways involving hemolysis-associated defects in nitric oxide bioavailability, oxidative stress, ischemia-reperfusion injury, hemostatic activation, leukocytes and platelets. Vasculopathy of sickle cell disease has been implicated in the development of pulmonary hypertension, stroke, leg ul

SUBMITTER: Kato GJ 

PROVIDER: S-EPMC3209715 | biostudies-literature | 2009 Sep

REPOSITORIES: biostudies-literature