Severe jaundice due to coexistence of Dubin-Johnson syndrome and hereditary spherocytosis: A case report

Dubin-Johnson syndrome is a chronic, benign, intermittent jaundice, mostly of conjugated hyperbilirubinemia. The level of biliru- bin is not expected to be more than 20 mg/dl in this syndrome. In this article, we report a patient who was evaluated for hyperbili- rubinemia and liver function test abnormalities and diagnosed with Dubin-Johnson syndrome coexisting with hereditary spherocy- tosis. We suggest that other diseases should be investigated if patients with Dubin-Johnson syndrome present with severe hyperbi- lirubinemia. Dubin-Johnson syndrome accompanied by hemolytic diseases might also have high coproporphyrin levels (as in Ro- tor's syndrome) than expected in pure Dubin-Johnson syndrome.