Abstract: Objective To investigate the clinical and pathological features of Dubin-Johnson syndrome (DJS) in 21 cases.Methods Clinical data of 21 inpatients diagnosed of DJS from January 2006 to April 2016 was collected and retrospectively analyzed.Results There were 17 male patients and 4 female patients enrolled with average age of 28.7±8.1-year old. The average levels of total bilirubin (TBil) and direct bilirubin were 59.2±11.1 mol/L and 40.9±9.3 mol/L, respectively. Macroscopic observation shown liver tissues with pathognomonic black including gray-brown, yellow-green and gray-black. Microscopically, there were a large number of dark brown coarse granules depositing in the hepatocytes, especially around the central veins. A few of hydropic degeneration of hepatocytes was detected with little perisinuitis. There was no or slight enlargement in the portal areas with little inflammatory cells infiltration.Conclusion DJS occurs mainly in men during the phase of adolescence. Total bilirubin is increased in DJS, especially the direct bilirubin. Liver biopsy is the golden index for diagnosis of DJS, which is shown as grossly dark liver specimen and microscopically numerous brownish pigment deposits in hepatocytes.

Key words: Dubin-Johnson syndrome, Clinical feature, Pathology