Leg ulcers in sickle cell disease.
Sickle cell disease is a single amino acid molecular disorder of hemoglobin leading to its pathological polymerization, red cell rigidity that causes poor microvascular blood flow, with consequent tissue ischemia and infarction. The manifestations of this disease are protean.Among them, leg ulcers represent a particularly disabling and chronic complication, often associated with a more severe clinical course.Despite the fact that this complication has been recognized since the early times of SCD, there has been little improvement in the efficacy of its management and clinical outcome over the past 100 years. Recently, vasculopathic abnormalities involving abnormal vascular tone and activated, adhesive endothelium have been recognized as another pathway to end organ damage in sickle cell disease. Vasculopathy of sickle cell disease has been implicated in the development of pulmonary hypertension, stroke, leg ulceration and priapism, particularly associated with hemolytic severity, and reported in other severe hemolytic disorders. The authors present the proceedings from the Educational Session on Chronic leg ulcers in Sickle cell disease, held during the 4th Annual Sickle Cell Disease Research and Educational Symposium, on February 17, 2010 in Fort Lauderdale, Fla.
Minniti
Caterina P
CP
Pulmonary Vascular Medicine Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892, USA. minnitic@mail.nih.gov
Eckman
James
J
Sebastiani
Paola
P
Steinberg
Martin H
MH
Ballas
Samir K
SK
D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000503:physiopathology; D003131:Combined Modality Therapy; D004730:Endothelium, Vascular / Q000473:pathology / Q000503:physiopathology; D005260:Female; D056726:Genetic Association Studies; D020022:Genetic Predisposition to Disease; D006453:Hemoglobinopathies / Q000150:complications / Q000503:physiopathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000453:epidemiology / Q000209:etiology; D007511:Ischemia / Q000209:etiology; D007866:Leg / Q000098:blood supply; D007871:Leg Ulcer / Q000145:classification / Q000453:epidemiology / Q000209:etiology* / Q000235:genetics / Q000517:prevention & control / Q000628:therapy; D008297:Male; D008875:Middle Aged; D012720:Severity of Illness Index