Knowledge Check

Hereditary spherocytosis is a disorder of:

a
Hemoglobin
b
Red cell membrane
c
Red cell enzyme

Hemolysis in hereditary spherocytosis is typically:

a
Intravascular
b
Extravascular
Spherocytes are prone to hemolysis. The mechanisms include reduced deformability, which impairs passage through constricted regions of the microcirculation, and phagocytosis by splenic macrophages, which occurs in response to splenic trapping.

True or false: hereditary spherocytosis is associated with a wide clinical spectrum.

a
True
b
False

True or false: splenectomy is effective at improving anemia in severe cases of hereditary spherocytosis.

a
True
b
False
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