期刊
CLINICAL HEMORHEOLOGY AND MICROCIRCULATION
卷 68, 期 2-3, 页码 173-186出版社
IOS PRESS
DOI: 10.3233/CH-189006
关键词
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资金
- National Institutes of Health, National Heart Lung and Blood Institute [5 RC1 HL099412-01, K12 HD52954-6 A1, 1 K23 HL 119627-01A1, 1 R03 HL 138321-01]
- Children's Hospital Los Angeles General Clinical Research Center (NIH) [RR00043-43]
Sickle cell anemia is characterized by a mutation resulting in the formation of an abnormal beta-hemoglobin called hemoglobin S. Hemoglobin S polymerizes upon deoxygenation, causing impaired red blood cell deformability and increased blood viscosity at equivalent hematocrits. Thus, sickle cell disease is a hemorheologic disease that results in various pathologic processes involving multiple organ systems including the lungs, heart, kidneys and brain. Red blood cell mechanics and the perturbations on blood flow-endothelial interaction underlie much of the pathology found in sickle cell disease. Transfusion therapy is one of the few therapeutic options available to patients, acting as both primary and secondary prevention of stroke. Transfusion therapy, both simple and exchange, is also used for unremitting and frequent pain crises and pulmonary hypertension. Therefore, understanding basic rheologic changes following transfusion inform other therapeutic options that aim to mitigate this diffuse pathologic process. This review will aim to highlight transfusion effects on blood rheology.
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