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The pathophysiology of sickle cell disease: from the genetic mutation to multiorgan disfunction. The most important pathophysiological abnormalities of sickle cell disease are reviewed, taking into account three levels: a molecules and cells, b tissues, c organism as a whole. We dedicated especial attention to the chronic inflammatory phenomena, abnormally expressed adhesion molecules, the interaction among sickle cells, endothelial cells, leukocytes and platelets, and their role in the pathophysiology of vaso-occlusion.
The clinical manifestations, like anemia, pain crises and multiorgan dysfunction are covered. Factors that modulate disease severity, like the co-inheritance of a-thalassemia, gene mutations related to thrombophilia and the elevation of fetal hemoglobin are discussed. Key words: Sickle cell disease; pathophysiology; inflammation; adhesion molecules.
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O tema apresentado e o convite ao s autor es constam da pauta elaborada pelo co-editor, prof. All the contents of this journal, except where otherwise noted, is licensed under a Creative Commons Attribution License. Services on Demand Journal. ABSTRACT The most important pathophysiological abnormalities of sickle cell disease are reviewed, taking into account three levels: a molecules and cells, b tissues, c organism as a whole.
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