title: Haemolytic anaemia as first manifestation of Wilson's disease: a report of two cases.
abstract: Wilson's disease can have different manifestations like jaundice, cirrhosis of liver, extrapyramidal symptoms and dementia. Haemolytic anaemia may occur but it is commonly associated with florid manifestation of liver disease. Sometimes, liver cell necrosis can release huge free copper ions in blood, giving rise to oxidant damage to erythrocytes. Oxidative damage to cell membrane, haemoglobin and erythrocyte metabolism causes haemolytic crisis. In some cases, liver involvement is subclinical, but nonetheless, free copper is released from necrosed hepatocytes and causes oxidative damage to erythrocytes.We had two cases of Wilson's disease with initial presentation as severe haemolytic anaemia and no other clinical feature suggestive of Wilson's disease was present. In unclear cause of haemolytic anaemia, especially in adolescents or young adults, Wilson's disease should be considered. As Wilson disease is rare and its initial presentation with haemolytic anaemia is rarer, high level of suspicion is required to diagnose it.
authors.forename: Gouranga
authors.initials: G
authors.lastname: Santra
authors.forename: Rudrajit
authors.initials: R
authors.lastname: Paul
authors.forename: Partha Sarathi
authors.initials: PS
authors.lastname: Choudhury
authors.forename: Sumit Kr
authors.initials: SK
authors.lastname: Ghosh
authors.forename: Dibyendu
authors.initials: D
authors.lastname: De
authors.forename: Shubhabrata
authors.initials: S
authors.lastname: Das
mesh_terms: D000293:Adolescent; D000743:Anemia, Hemolytic / Q000209:etiology*; D006527:Hepatolenticular Degeneration / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D008297:Male; D008875:Middle Aged