Spherocytosis

Spherocytosis is an inherited or acquired disease of erythrocytes, in which they take a spherical, rather than the normal flattened disc form. In the spherical form, they are more prone to breakdown and thus producing hemolytic anemia.

Hereditary spherocytosis is most common. The disease also can be secondary to infections, including infectious mononucleosis; and hepatolenticular degeneneration (Wilson's disease).

Mechanism

The abnormal cells have a surface protein that marks them for early destruction in the spleen. Splenectomy, therefore, is a treatment.

Diagnosis

Examination of the erythrocytes will show an increase in red blood cell width (anisocytosis); the smaller cells will be spherocytic.