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Extracorpuscular non-immune hemolytic anemia: Fragmentation hemolysis and hypersplenism

Stanley L Schrier, MD
Section Editor
William C Mentzer, MD
Deputy Editor
Jennifer S Tirnauer, MD


Hemolytic anemia is defined as a shortening of red blood cell (RBC) survival to less than 100 days (normal: approximately 120 days) due to an increased rate of destruction of RBCs. (See "Diagnosis of hemolytic anemia in the adult".)

Hemolytic anemia is caused by one of two mechanisms:

Abnormalities arising from within the RBC and/or its membrane (intracorpuscular defects). These are usually congenital (eg, sickle cell anemia, thalassemia, glucose-6-phosphate dehydrogenase (G6PD) deficiency, hereditary spherocytosis), but may rarely be acquired, as in paroxysmal nocturnal hemoglobinuria.

Abnormalities in the environment of the RBC. These processes are, by definition, extracorpuscular, and the ensuing hemolysis is called extrinsic hemolytic anemia.

Extracorpuscular hemolytic anemia is often due to immune mechanisms, as in warm and cold agglutinin hemolytic anemia. However, there are many nonimmune causes, including systemic diseases, mechanical destruction (ie, fragmentation hemolysis), increased destruction within an enlarged spleen (hypersplenism), and the action of toxins and drugs. (See "Diagnosis of hemolytic anemia in the adult", section on 'Conceptual framework'.)

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Literature review current through: Nov 2017. | This topic last updated: Sep 12, 2017.
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