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The formation of spherocytes occurs due to the defects in the vertical protein linkages between the membrane and cytoskeleton, resulting in a loss of unsupported rbc membrane and spherocyte formation Hereditary spherocytosis results from defects in the structural proteins of the red blood cell (rbc) membrane, particularly spectrin, ankyrin, and band 3 protein. Spherocytosis most often refers to hereditary spherocytosis
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This is caused by a molecular defect in one or more of the proteins of the red blood cell cytoskeleton, including spectrin, ankyrin, band 3, or protein 4.2. It is a genetically and clinically heterogeneous disorder of the rbc membrane characterized by defective vertical interactions between the lipid bilayer and the cytoskeleton of the rbc membrane, resulting in increased vesiculation and loss of rbc membrane. Molecular defects in these proteins decrease membrane integrity, leading to vesiculation, decreased membrane surface area, and reduced deformability of the cells
Eventually, this leads to the trapping the abnormal rbcs (spherocytes) in the spleen.
This anemia happens when your red blood cells break down faster than normal The underlying defect is the progressive loss of surface membrane without a similar loss of volume This causes the erythrocyte shape to change from one with a very high surface area to volume ratio (the biconcave disc) to a cell with minimum surface area to volume ratio (a sphere). Spherocytosis are cells that lack central pallor and are spherical in shape (they have lost their membrane without losing the cytosol).
Spherocytes (figs 1 and 2) result from loss of membrane in excess of loss of cytoplasm, either through the action of macrophages of the reticuloendothelial system or through fragmentation in the circulation. In hereditary spherocytosis, there is a lack of spectrin, a key rbc cytoskeletal membrane protein
