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Extrinsic nonimmune hemolytic anemia due to systemic disease

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

INTRODUCTION

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"). This process is caused by one of two mechanisms:

Abnormalities arising from within the RBC and/or its membrane (intracorpuscular defects). One can think of the RBC as having three compartments: hemoglobin, metabolic machinery to energize ion pumps and provide requisite reducing power, and a deformable elastic membrane. Abnormalities of these three compartments 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 (PNH).

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

Extrinsic hemolytic anemia is often due to immune mechanisms, as in warm and cold agglutinin hemolytic anemia. (See "Diagnosis of hemolytic anemia in the adult", section on 'Conceptual framework'.) 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.

The systemic disorders associated with nonimmune extrinsic hemolytic anemia will be reviewed here, including infection, liver and renal diseases, and miscellaneous conditions such as anorexia nervosa. Extrinsic hemolysis due to fragmentation hemolysis, hypersplenism, and oxidative hemolysis are discussed separately. (See "Extrinsic nonimmune hemolytic anemia due to mechanical damage: Fragmentation hemolysis and hypersplenism" and "Hemolytic anemia due to drugs and toxins".)

                          

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Literature review current through: Nov 2016. | This topic last updated: Thu Oct 08 00:00:00 GMT+00:00 2015.
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