Hereditary spherocytosis pathophysiology: Difference between revisions
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==Overview== | ==Overview== | ||
There is intrinsic defects in erythrocyte membrane proteins that result in RBC cytoskeleton instability. Loss of erythrocyte surface area leads to the spherical shape of RBCs (spherocytes), which are culled rapidly from the circulation by the spleen. Hemolysis mainly confined to the spleen and, therefore, is extravascular. Splenomegaly commonly develops. | |||
==Pathophysiology== | ==Pathophysiology== | ||
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Revision as of 14:13, 2 July 2018
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
There is intrinsic defects in erythrocyte membrane proteins that result in RBC cytoskeleton instability. Loss of erythrocyte surface area leads to the spherical shape of RBCs (spherocytes), which are culled rapidly from the circulation by the spleen. Hemolysis mainly confined to the spleen and, therefore, is extravascular. Splenomegaly commonly develops.