Clinical features
Hereditary spherocytosis may present with jaundice in childhood or may be discovered in later life, often incidentally. Most patients develop a partially compensated haemolytic state characterised by:
- anaemia - variable, dependent upon the degree of bone marrow compensation
- splenomegaly
- mild or intermittent jaundice, caused by haemolysis. Occassionally it may present as severe jaundice in the neonatal period.
- pigment gallstones, secondary to jaundice
- attacks of cholecystitis
- occasionally, leg ulceration
Additionally, periods of severe anaemia may arise - aplastic crises, precipitated by infection, often by human parvovirus, or folate deficiency, especially in pregnancy.
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