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Hereditary Elliptocytosis
It has a similar incidence to HS.
Autosomal dominant inheritance.
Symptoms
Symptoms are identical to HS, they range from asymptomatic to severe.
Abnormality revolves around spectrin leading to skeletal changes which
can cause the cell to fragment
Asymptomatic group (majority)
Haemolytic anaemia group
Haematological Profile
More than 25% elliptocytes are required for a diagnosis of
elliptocytosis
Because erythropoiesis is compensated for, polychromasia is present
Treatment
Splenectomy, if more than 5 years old (corrects anaemia due to
haemolysis, still more susceptible to infection (vaccination becomes
even more essential)) and the haemolysis is severe.
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