What mechanism leads to hemolysis in hereditary spherocytosis?

In hereditary spherocytosis, the primary mechanism leading to hemolysis is the decreased lifespan of red blood cells. This condition is characterized by a defect in the proteins that make up the erythrocyte membrane, primarily spectrin and ankyrin. These proteins maintain the biconcave shape of red blood cells, which is essential for their flexibility and stability as they navigate through small blood vessels in the spleen and elsewhere.

Due to the membrane defects, affected red blood cells assume a spherical shape instead of the normal disc shape. This increased rigidity reduces their ability to deform when traversing narrow passages, particularly in the spleen, where these cells are filtered out and destroyed. As a result, the lifespan of red blood cells in individuals with hereditary spherocytosis is significantly decreased, leading to a hemolytic anemia characterized by the premature breakdown of these cells.

While increased hemoglobin production, excessive iron absorption, and an increased leukocyte response are important concepts in hematology, they do not directly relate to the underlying mechanism of hemolysis in hereditary spherocytosis. The pathology is specifically linked to the structural abnormalities of the red blood cell membrane and the consequent reduced lifespan of the affected cells.