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Scientific literature and patient education texts
Facts About Sickle Cell Disease
source: Novartis Pharmaceuticals Corporation
year: 2019
summary/abstract:Sickle Cell Disease Goes Beyond Red Blood Cells and, Early on, Progresses to a Chronic Vascular Disease Vaso-Occlusive Crises May Account for a Majority of the Burden of Sickle Cell Disease
• Sickle cell disease (SCD) is a genetic blood disorder arising from mutations in the hemoglobin gene
• Multicellular adhesion among endothelium and blood cells is a major driver of vaso-occlusion and vaso-occlusive crises (VOCs)
• The upregulation and expression of specific adhesion mediators, including selectins, drive multicellular adhesion clusters
• VOCs are the clinical hallmark of SCD and originate from ongoing, silent, vaso-occlusion
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