Scuola di Specializzazione in Pediatria, IRCCS Materno-Infantile “Burlo Garofolo”, Università di Trieste
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Key words: Spherocytosis, Anaemia, Splenomegaly
Summary Hereditary spherocytosis is a common inherited disorder that is characterized by anaemia, jaundice and splenomegaly. It is reported worldwide and is the most common inherited anaemia in individuals of northern European ancestry. Clinical severity is variable with most of patients having a wellcompensated haemolytic anaemia. Some individuals are asymptomatic, whereas others have severe haemolytic anaemia requiring erythrocyte transfusion. Common complications are cholelithiasis and aplastic crises. Splenectomy is curative and it is indicated in children with moderately-severe and severe hereditary spherocytosis and in patients with symptoms of cholelithiasis.
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