Alteration of the erythrocyte membrane usually signals the reticuloendothelial macrophages to remove the damaged red blood cell (RBC) from the circulation. In extraordinary circumstances, however, the damage to the membrane is so great that the erythrocyte undergoes hemolysis, and its intracellular contents, including hemoglobin, are liberated into the plasma. This chapter describes the structural and functional features of normal erythrocytes and diseases involving membrane architecture, RBC proteins, and extracorpuscular factors that can lead to shortened RBC survival. The chapter contains major discussions of sickle cell disease and the thalassemias. Included are tables providing information on erythrocyte metabolism and etiologies of hemolysis in glucose-6-phosphate dehydrogenase (G6PD) deficiency; figures illustrating histologic features of abnormal erythrocytes and sickle cells are also provided. This review contains 7 figures, 2 tables, and 243 references.
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