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Patients with sickle cell disease (i.e., with hemoglobin [Hb] genotype SS) have episodes of pain crises, which are attributed to obstruction of microcirculation by irreversibly sickled erythrocytes adhering to the endothelium. However, occlusion of large vessels also can occur, leading to infarction of various organs, including the brain, liver, spleen, and kidney. Pulmonary hypertension is another complication of sickle cell disease and is due to obliterative pulmonary vasculopathy, characterized by intimal hyperplasia, fibrosis, and thrombosis (N Engl J Med 2004; 350:857). In addition, pulmonary emboli often are identified in imaging evaluations of patients with hemoglobinopathies, including patients with sickle cell disease or sickle cel…