Figure 46

Polymerization of hemoglobin S. Polymerization of deoxygenated hemoglobin S is the primary event in the molecular pathogenesis of sickle cell disease, resulting in a distortion of the shape of the erythro-cyte and a marked decrease in its deformability. These rigid cells are responsible for the vaso-occlusive phenomena that are the hallmark of the disease [4]. Interesting shapes of variable forms result depending on the localization of the polymers in the cell. A collection of electron microscopy scans of sickle cells undergoing intracellular polymerization is shown here. The slides were created in different laboratories. A, Characteristic peripheral blood smear from a patient with sickle cell anemia. Extreme sickled forms and target cells are seen. B, Electron microscopy scan of normal erythrocytes.

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