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Spherocytic hereditary elliptocytosis accounts for 10% of cases hereditary elliptocytosis and related disorders. Elliptocytes are less prominent and more spheroidal than the typical mild condition, and may be dominated by spherocytes. Clear cut elliptocytosis is seen in at least one family member.
The clinical presentation resembles that of hereditary spherocytosis with patients typically having moderate haemolysis, mild anaemia and poikilocytosis.
Most respond well to splenectomy.
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