27例原发性胆汁性肝硬化的组织病理学特征

Histppathological features of 27 cases of primary biliary cirrhosis

目的 总结原发性胆汁性肝硬化（PBC）患者的临床及病理组织学特点。方法 对27例原发性胆汁性肝硬化患者的临床资料进行分析,重点讨论其肝脏组织病理学特点。结果 本组男、女之比为1:8（3:24）,年龄22～69岁。其主要临床症状为乏力（62.9％,17/27）,其次为黄疸（59.2％,16/27）和皮肤瘙痒（29.6％,8/27）。患者的血清碱性磷酸酶（ALP）及γ-谷氨酰转肽酶（GGT）均明显升高,95.8％的患者（23/27）抗线粒体抗体或线粒体抗体M2亚型阳性。肝组织病理学特点为:小叶间胆管变性坏死、基底膜不完整,周围有淋巴细胞和浆细胞浸润（66％,18/27）;汇管区淋巴细胞聚集（10％,27/27）或淋巴滤泡形成（15％,4/27）;肉芽肿形成（26％,7/27）及小叶间胆管减少;细小胆管增生（55％,15/27）,肝细胞羽毛状变性（59％,16/27）;肝细胞内胆色素沉积和（或）毛细胆管胆栓形成（52％,14/27）;纤维组织增生,小叶结构紊乱（26％,7/27）,假小叶形成（11％,3/27）。结论 乏力、皮肤瘙痒及血清ALP、GGT升高及抗线粒体抗体阳性是PBC的主要临床特征;而小叶间胆管炎、胆管数目减少,汇管区淋巴细胞聚集、肉芽肿形成、细小胆管增生,以及肝细胞羽毛状变性是PBC的主要病理特点。

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 γ-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, markerdly elevated phosphatase and γ-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 proliferatioin in the portal area; and (3)feathery degeneration of hepatocytes.