Hereditary spherocytosis is an autosomal dominant defect of the RBC membrane leading to the formation of extremely vulnerable, defective RBCs known as spherocytes. These cells are prematurely destroyed in the spleen, leading to hemolytic anemia.
Characteristics
- Most common hereditary hemolytic anemia in Northern Europeans.
- Parvovirus B19 can exacerbate the anemia by causing aplastic crisis.
Pathophysiology
The defect is usually in ankyrin or spectrin, which are structural proteins, leading to