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[Histopathological features of 27 cases of primary biliary cirrhosis]
Xiaoyan Shi1, Changhuai Zhang, Jidong Jia
1Beijing Friendship Hospital, Capital University of Medical Sciences, Beijing 100050, China.
Objective:
To explore the clinical and histopathological features of primary biliary cirrhosis (PBC).
Methods:
The clinical, laboratory, as well as histological features of 27 cases of PBC were retrospectively analyzed.
Results:
The male to female ratio was 1:8 (3:24), aged from 22 to 69 years. The main clinical manifestations included: fatigue (62.9%, 17/27), jaundice (59.2%, 16/27) and pruritus (29.6%, 8/27), with all of patients having markedly elevated serum alkaline phosphatase and gamma-glutamyl transpeptidase and 95.8% (23/27) of the patients being positive for anti-mitochondrial antibody (AMA). The main histopathological changes were: necroinflammation of interlobular bile ducts (100%, 27/27), lymphocyte aggregation or lymphocyte follicles (15%, 4/27), granuloma (26%, 7/27), decreased number of interlobular bile ducts and smaller bile duct proliferation (55%, 15/27), feathery degeneration of hepatocytes (59%, 16/27); bilirubinostasis in hepatocytes and/or canaliculi (52%, 14/27); fibrosis and distortion of lobules (26%, 7/27), pseudolobular formation (11%, 3/27).
Conclusions:
The main clinical features of PBS are fatigue and pruritus, markedly elevated phosphatase and gamma-glutamyl transpeptidase, and positive AMA with or without jaundice. Its histopathological hallmarks are (1)necroinflammmation and ductopenia involved mainly in interlobular bile ducts; (2)lymphocyte aggregation, granuloma formation and bile ductular proliferation in the portal area; and (3)feathery degeneration of hepatocytes.