Disable ads (and more) with a premium pass for a one time $4.99 payment
Hereditary spherocytosis is a condition characterized by the production of abnormally shaped red blood cells that are spherical rather than the typical biconcave disc shape. These spherocytes are more fragile and prone to lysis. The hemolysis in hereditary spherocytosis predominantly occurs extravascularly, primarily within the spleen.
In this condition, the spleen recognizes these abnormal spherocytes as defective and removes them from circulation. Because this process takes place outside of the blood vessels, it's classified as extravascular hemolysis. This aligns with the typical clinical presentation of hereditary spherocytosis, which often includes symptoms of anemia, jaundice, and splenomegaly due to increased destruction of these spherocytes in the spleen.
Other types of hemolysis, such as intravascular hemolysis, occur when red blood cells rupture within the bloodstream, often due to mechanical trauma or immune-mediated destruction, which is not the primary mechanism at play in hereditary spherocytosis. Hence, extravascular hemolysis is the correct classification for the hemolytic process observed in individuals with hereditary spherocytosis.