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Table 62-3

Classification of Hemolytic Anemiasa

Intracorpuscular DefectsExtracorpuscular Factors
Hereditary

Hemoglobinopathies

Enzymopathies

Membrane-cytoskeletal defects

Familial (atypical) hemolytic uremic syndrome
AcquiredParoxysmal nocturnal hemoglobinuria (PNH)

Mechanical destruction (microangiopathic)

Toxic agents

Drugs

Infectious

Autoimmune

aHereditary causes correlate with intracorpuscular defects because these defects are due to inherited mutations. The one exception is PNH because the defect is due to an acquired somatic mutation. Similarly, acquired causes correlate with extracorpuscular factors because mostly these factors are exogenous. The one exception is familial hemolytic uremic syndrome (HUS; often referred to as atypical HUS) because here an inherited abnormality allows complement activation to be excessive, with bouts of production of membrane attack complex capable of destroying normal red cells.