Pronicki Maciej
Department of Pathology, The Children's Memorial Health Institute, Warsaw, Poland.
Handb Clin Neurol. 2017;142:71-75. doi: 10.1016/B978-0-444-63625-6.00007-0.
The liver in Wilson disease may demonstrate a wide range of damage patterns. Some patients may present almost no detectable microscopic pathology, while others display lesions consistent with fulminant hepatitis or acute liver failure. Most liver biopsy specimens show moderate to severe steatosis, variable degree of portal and/or lobular inflammation, and fibrosis eventually progressing to cirrhosis. Additional findings include liver cell degeneration and ballooning, Mallory hyaline bodies, liver cell necrosis, and glycogenation of periportal hepatocytic nuclei. None of the above lesions are specific for Wilson disease and should be interpreted in a wider medical context and particular clinical setting. The main message concerning liver pathology is that Wilson disease may be microscopically misinterpreted as many other liver diseases, including viral or autoimmune hepatitis, alcoholic/nonalcoholic steatohepatitis, toxic liver injury, cryptogenic cirrhosis, metabolic liver disease, and many others. The possibility of Wilson disease should be considered in all patients, especially young ones presenting unexplained liver diseases with many variable patterns of microscopic liver involvement.
威尔逊病患者的肝脏可能呈现多种损伤模式。一些患者可能几乎没有可检测到的微观病理变化,而另一些患者则表现出与暴发性肝炎或急性肝衰竭一致的病变。大多数肝活检标本显示中度至重度脂肪变性、不同程度的门脉和/或小叶炎症,以及最终发展为肝硬化的纤维化。其他发现包括肝细胞变性和气球样变、马洛里透明小体、肝细胞坏死以及门周肝细胞核的糖原化。上述病变均非威尔逊病所特有,应在更广泛的医学背景和特定临床环境中进行解读。关于肝脏病理学的主要信息是,威尔逊病在显微镜下可能被误诊为许多其他肝脏疾病,包括病毒性或自身免疫性肝炎、酒精性/非酒精性脂肪性肝炎、中毒性肝损伤、隐源性肝硬化、代谢性肝病等等。所有患者,尤其是那些出现原因不明的肝脏疾病且微观肝脏受累模式多样的年轻患者,都应考虑威尔逊病的可能性。