Hereditary Spherocytosis

Question 1

What gender is commonly affected by hereditary spherocytosis?

Answer 1

Male + females affected equally

Question 2

Inheritance pattern

Answer 2

autosomal dominant

Question 3

What ethnicity is most prone to hereditary spherocytosis?

Answer 3

Northern European descent

Question 4

Typical clinical features in hereditary spherocytosis

Answer 4

• Neonatal jaundice
• Haemolytic crises precipitated by infection
• Gallstones
• Splenomegaly

Question 5

Finding on blood film in HS

Answer 5

Spherocytes (round, lack of central pallor)

Question 6

Diagnostic test for HS

Answer 6

EMA binding test