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Polymerization of deoxyhemoglobin S within sickle erythrocytes is of cardinal importance to each of the clinical complications of sickle cell anemia. Sickle gene expression, however, is modulated by a number of pathophysiological mechanisms that generate vast clinical diversity among sickle cell patients. These processes include genotypic variations, modifi cations of polymerization, abnormal erythrocyte hydration, membrane defects, and rheologic factors. In many cases it is possible to relate specific pathophysiological mechanisms to particular disease features or to possi bilities for therapeutic intervention. INTRODUCTION The clinical manifestations of sickle cell anemia are due ultimately to the poor solubility and intraerythrocytic polymerization of deoxygenated hemoglobin S, the product of inherited sickle cel\ genes (1, 2). It is enigmatic that this seemingly straightforward etiology results in tremendous clinical diversity among sickle cell patients (3). Estimates of disease severity based strictly on predictions of the degree of polymerization apply mainly to the level of chronic hemolytic anemia (4). A more comprehensive understand ing of the broader spectrum of disease features is achieved by considering the genetic, molecular, cellular, and rheologic mechanisms that modify sickle gene expression. 361 0066-4219/86/0401-0361$02.00 EMBURY The purpose of this review is to describe the pathophysiologic mechan isms that mediate
Annual Review of Medicine – Annual Reviews
Published: Feb 1, 1986
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