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

## TL;DR

A man with hereditary spherocytosis experienced severe jaundice and cholestasis with no clear cause, which resolved on its own.

## Contribution

This case highlights a rare and unexplained association between hereditary spherocytosis and acute cholestasis.

## Key findings

- The patient had severe hyperbilirubinaemia and cholestasis with no identifiable cause.
- Liver biopsy confirmed acute severe cholestasis.
- The condition resolved spontaneously without targeted treatment.

## Abstract

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.