Classification of Hemolytic Anemiasa
Intracorpuscular Defects | Extracorpuscular Factors | |
---|---|---|
Hereditary | Hemoglobinopathies Enzymopathies Membrane-cytoskeletal defects | Familial (atypical) hemolytic uremic syndrome |
Acquired | Paroxysmal 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.