Case 63 – update 4

Our four year old appears to have acute parvovirus infection. It commonly causes reduced rd cell production but this is generally only significant in patients with increased red cell destruction, who rely on a very active marrow e.g. inherited and acquired haemolytic anaemias.

 

He has spherocytes on his blood film pointing towards hereditary spherocytosis. Autoimmune haemolysis can also cause spherocytosis but this would be unusual in this age group and there is a negative DAT. It transpires his father had a splenectomy when he was a child but is not sure why. The patient also had mildly prolonged jaundice at birth but was not investigated further. An EMA (eosin-5′-maleimide) binding test is performed and this shows reduced binding consistent with hereditary spherocytosis

 

 

Questions

  • How is hereditary spherocytosis managed?
  • How is hereditary spherocytosis inherited?
  • What are the long-term complications?

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