Histopathological features of 27 cases of primary biliary cirrhosis / 中华肝脏病杂志

ABSTRACT

<p><b>OBJECTIVE</b>To explore the clinical and histopathological features of primary biliary cirrhosis (PBC).</p><p><b>METHODS</b>The clinical, laboratory, as well as histological features of 27 cases of PBC were retrospectively analyzed.</p><p><b>RESULTS</b>The male to female ratio was 18 (324), 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).</p><p><b>CONCLUSIONS</b>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.</p>