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Updated: Feb 17, 2026

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Transient Unexplained Severe Acute Hyperbilirubinaemia and Cholestasis in a Patient With Hereditary Spherocytosis

Jennifer Richardson1, Michael Johnston2, Alison Sefcick3

  • 1IMT Trainee West of Scotland, Queen Elizabeth University Hospital Glasgow, NHS Greater Glasgow and Clyde, Glasgow, UK, nhsggc.org.uk.

Case Reports in Hepatology
|February 16, 2026
PubMed

Hereditary spherocytosis is an inherited red cell membrane disorder resulting in haemolytic anaemia. Recognised clinical manifestations include anaemia, jaundice, splenomegaly and gallstones. Here we describe the case of a 40-year-old male with hereditary spherocytosis presenting with severe hyperbilirubinaemia. Liver biopsy demonstrated features consistent with acute severe cholestasis. Despite extensive investigations for gallstone disease and other causes of liver pathology, no aetiology was identified. There are very few reports in the literature describing cases of profound unexplained jaundice in hereditary spherocytosis. Hereditary spherocytosis may be associated with idiopathic acute cholestasis. We report that the case was managed conservatively and spontaneously resolved.

Keywords:
acute cholestasishaematologyhepatologyhereditary spherocytosis

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