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A 15 year old girl with fever, jaundice, haemolysis, and sudden clinical deterioration

Q1: What does the liver biopsy show (see p 250)?
A portal triad with marked oedema and fibrosis extending towards other portal triads is shown in fig 1A. Focal mononuclear cell infiltrates, ductular proliferation, and interface hepatitis are also visible. In fig 1B, hepatocyte ballooning and lytic necrosis are associated with feathery degeneration.

These morphological findings are insufficient by themselves to establish a diagnosis since they are shared by a number of different liver diseases.

Q2: What is the most likely diagnosis?
The differential diagnosis in this case must include all the most important causes of fulminant hepatic failure (box 1). Nevertheless, fulminant Wilson's disease should be considered the leading diagnosis. The three most relevant clues pointing to Wilson's disease are the age of the patient, the presence of Coombs negative haemolytic anaemia, and the low serum alkaline phosphatase level. Liver biopsy findings are compatible with Wilson's disease, since the vast majority of Wilson's disease patients have evidence of fibrosis, despite widely varying levels and patterns . . . [Full text of this article]

Relevant Article

A 15 year old girl with fever, jaundice, haemolysis, and sudden clinical deterioration

M Pirisi, B Branca, C Avellini, and A Solinas
Postgrad. Med. J. 2002 78: 250. [Extract] [Full Text] [PDF]