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How does hereditary spherocytosis change the shape and survival of red blood cells?
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blood disease
Peripheral blood smear revealing histopathologic features indicative of a blast crisis in a case of chronic myelogenous leukemia (CML). Blast crisis occurs when one-fifth to one-third of the cells in the blood or bone marrow are immature blood cells, or blast cells. This phase of CML is characterized by fever, weakness, and an enlarged spleen.
Hereditary spherocytosis is a congenital disorder that fundamentally alters the structure of red blood cells by weakening the cell membrane. Instead of their normal disk-like shape, these cells become spherical and variable in size.12
This structural change significantly impacts cell survival in the following ways:
- Mechanical Fragility: The spherical cells are inherently fragile and prone to bursting when exposed to dilute salt solutions.12
- Metabolic Stress: Because of their shape, these cells are often deprived of adequate access to plasma glucose, which is essential for their energy needs.2
- Splenic Sequestration: The abnormal spheroidal shape causes the cells to remain in the spleen longer than healthy cells. The spleen acts as a site of destruction, where these trapped, fragile cells are broken down, leading to chronic hemolytic anemia.21
While the condition is lifelong, the removal of the spleen (splenectomy) is often effective in curing the anemia by eliminating the primary site where these defective cells are sequestered and destroyed.12