Li Melissa K, Crawford James M
University of Florida College of Medicine, Gainesville, Florida 32610-0275, USA.
Semin Liver Dis. 2004 Feb;24(1):21-42. doi: 10.1055/s-2004-823099.
Hepatic formation of bile is critical to survival and is one of the most easily disrupted liver functions. Liver biopsy is performed to obtain a definitive diagnosis of cause, to exclude potential etiologies, or simply to assist in development of a differential diagnosis. Parenchymal changes of cholestasis (feathery degeneration of hepatocytes, dilated bile canaliculi with retained bile, Kupffer cell phagocytosis of bile that has leaked into the sinusoidal space) are nonspecific and may be seen with both nonobstructive and obstructive cholestasis. The portal tract changes of obstruction are characteristic: bile ductular proliferation, inspissated bile in bile ducts, portal tract edema, neutrophilic inflammation, and cholate stasis of periportal hepatocytes. Uncorrected obstruction incites robust fibrogenesis by portal tract myofibroblasts, engendering a characteristic jigsaw pattern of fibrous septa. Diseases with specific histological features include primary biliary cirrhosis, primary sclerosing cholangitis, biliary atresia, and graft-versus-host disease. However, the pathologist is cautioned not to overinterpret the cholestatic liver biopsy and to apply rigorous criteria for specific causal diagnoses. Most of the histological features of cholestasis are nonspecific. Hence, both practicing physician and pathologist should have sound knowledge of the pathology of cholestasis.
肝脏胆汁生成对生存至关重要,且是最易受干扰的肝功能之一。进行肝活检以明确病因诊断、排除潜在病因,或仅仅是协助进行鉴别诊断。胆汁淤积的实质改变(肝细胞羽毛状变性、胆小管扩张伴胆汁潴留、库普弗细胞吞噬漏入窦状隙的胆汁)是非特异性的,在非阻塞性和阻塞性胆汁淤积中均可出现。阻塞性门管区改变具有特征性:胆小管增生、胆管内胆汁浓缩、门管区水肿、中性粒细胞炎症以及门周肝细胞胆酸盐淤积。未经纠正的阻塞会引发门管区肌成纤维细胞强烈的纤维生成,形成特征性的纤维间隔拼图样改变。具有特定组织学特征的疾病包括原发性胆汁性肝硬化、原发性硬化性胆管炎、胆管闭锁和移植物抗宿主病。然而,病理学家需注意不要过度解读胆汁淤积性肝活检结果,并应严格应用特定病因诊断标准。胆汁淤积的大多数组织学特征是非特异性的。因此,执业医师和病理学家都应具备扎实的胆汁淤积病理学知识。