Wilson’s disease (hepatolenticular degeneration) Wilson’s disease is an autosomal recessive disorder due to mutations in the gene ATP7B for copper-transporting ATPase located in the trans -Golgi network of the liver. It is uncommon but important and treatable. Normal hepatic copper transport is disrupted owing to various ATP7B mutations, leading to the accumulation of copper in hepatocytes and liver disease. The large number and diverse mutations identified…

Introduction Paediatric liver biopsies present a unique set of diagnostic problems for the pathologist, many of which become clinically apparent in the first few months of life as neonatal cholestasis . Among the important disorders one must consider in evaluating neonatal liver biopsies are extrahepatic biliary atresia , paucity of intrahepatic bile ducts (syndromatic and non-syndromatic types), metabolic diseases , viral hepatitis and the hepatic effects…

The hepatic arteries The effects of occlusion of hepatic artery branches are unpredictable because of the liver’s double blood supply and variable collateral flow. Potential effects of thrombotic or other occlusion include infarction and ischaemic damage to the biliary tree leading to stricture formation, cholangitis or duct rupture. The branches of the hepatic artery are sometimes involved in polyarteritis nodosa ( Fig. 12.1 ), the arteritis…

Introduction This chapter is intended to provide a working overview of the tumours and tumour-like nodular lesions that the pathologist will encounter with some frequency in everyday practice. The majority of these can be classified according to the putative cells of origin (hepatocytes, bile-duct epithelium and endothelium) from which they arise ( Table 11.1 ), and immunohistochemistry can often be used effectively to distinguish histogenesis. It…

Introduction Cirrhosis is a diffuse process in which the normal lobules are replaced by architecturally abnormal nodules separated by fibrous tissue. The nodules, which are most commonly the result of regenerative hyperplasia following hepatocellular injury, are functionally less efficient than normal hepatic parenchyma and there is a profound disturbance of vascular relationships. Several different kinds of information can be obtained about the cirrhotic liver by means…

Definition and causes Chronic hepatitis is a common reason for persistently abnormal liver function tests and forms the background for the development of much cirrhosis and hepatocellular carcinoma. It is defined as persistence of liver injury with raised aminotransferase levels or viral markers for more than 6 months. This definition, though artificial, helps to establish a borderline in studies of acute and chronic hepatitis. In practice,…

Introduction This chapter deals with the pathology of the important liver lesions attributed to drugs and toxins, with their recognition and with their differential diagnosis. There are hundreds of hepatotoxic drugs and other chemicals, and new reports of adverse drug reactions appear regularly in the literature under the acronym DILI (drug-induced liver injury). Heightened awareness of DILI during the last two decades has resulted in the…

Steatosis Steatosis (fatty change, fatty liver) is the accumulation of abnormal amounts of lipid in hepatocytes. Most steatosis is of the macrovesicular type, in which a single large fat vacuole or several smaller ones occupy the greater part of the cell, pushing the nucleus to the periphery ( Fig. 7.1 ). The less common and often more serious type is microvesicular steatosis ( Fig. 7.2 ).…

Introduction Acute hepatitis is not usually an indication for liver biopsy. There are, however, at least three reasons why pathologists sometimes receive liver biopsy samples from patients with acute hepatitis. First, there may be doubt about the clinical diagnosis, or even a mistaken working diagnosis. Second, a diagnosis of hepatitis may be well established but the clinician needs information on the stage of the disease or…

Introduction There are many sites along the biliary tree where bile flow may be interrupted, from the bile canaliculi and smallest intrahepatic ducts to the large bile ducts and duodenum ( Fig. 5.1 ). Damage or obstruction at these various sites may result in visible bile in histological sections (cholestasis), altered bile-duct morphology, changes within the portal tracts and periportal parenchyma, or combinations of these. Diseases…

Initial examination and reporting Naked-eye examination and description of biopsy specimens Although naked-eye examination and description are of limited diagnostic value, they reduce the possibility of specimen identification error. The pathologist should make sure that the whole specimen has been adequately sectioned by comparing the size of the sectioned and stained tissue with the measurement recorded on macroscopic examination. Naked-eye examination also helps in the selection…

Structures and components Functional units and nomenclature Under the low power of the light microscope, normal liver is seen to have a regular structure based on portal tracts and efferent veins. The smallest portal tracts contain portal venules, hepatic arterioles and small interlobular bile ducts. Blood from both venules and arterioles passes through the sinusoidal system to reach efferent hepatic venules. From these, the blood drains…

Processing of the specimen As soon as a needle biopsy specimen is obtained from the patient, it should be expelled gently into fixative or onto a piece of glass, card or wood. Filter paper is less suitable because fibres tend to adhere to the tissue and may interfere with sectioning. The specimen must be treated with great care, and excessive manipulation should be rigorously avoided; distortion…

Introduction Liver biopsy is one of many diagnostic tools used in the evaluation and management of patients with liver disease. It continues to play an important role because the concepts and classifications of liver disease are rooted in morphology. Moreover, looking at a liver biopsy specimen through the microscope is a very direct way of visualising the morphological changes that affect the liver in disease. The…