Page 645 - Textbook of Pathology, 6th Edition

is elevated which is transmitted to the liver via the inferior 2. Predominant elevation of aminotransferases (AST and 629
vena cava and hepatic veins. The patients generally have ALT).
enlarged and tender liver with mild liver dysfunction. 3. Hyperglobulinaemia (elevated IgG and γ-globulin).
Splenomegaly occurs due to simple passive congestion. 4. High serum titres of nuclear (ANA), smooth muscle (SMA),
and liver-kidney microsomal (LKM1) autoantibodies, and
MORPHOLOGIC FEATURES. Grossly, the liver is absence of antimitochondrial antibodies.
enlarged and firm with stretched Glisson’s capsule. 5. Concurrent presence of other autoimmune diseases.
Histologically, in acute stage, the hepatic sinusoids are 6. Presence of HLA DR3 or HLA DR4 markers.
dilated and congested with haemorrhagic necrosis of 7. Lack of prominent elevation of alkaline phosphatase.
centrilobular hepatocytes (central haemorrhagic necrosis). 8. Exclusion of chronic hepatitis of other known etiologies
Severe and more prolonged heart failure results in delicate (viral, toxic, genetic etc).
fibrous strands radiating from the central veins. These
fibrous strands may form interconnections leading to MORPHOLOGIC FEATURES. Autoimmune hepatitis is
cardiac cirrhosis and regenerative nodules. morphologically indistinguishable from chronic hepatitis
of viral etiology. Patients who survive active disease
Indian Childhood Cirrhosis develop crrhosis. There are features of burnt out chronic
autoimmune hepatitis accompanied with cirrhosis.
Indian childhood cirrhosis (ICC) is an unusual form of
cirrhosis seen in children between the age of 6 months and 3 Cirrhosis in Non-alcoholic Steatohepatitis
years in rural, middle class, Hindus in India and in parts of
South-East Asia and in the Middle-East. There is no role of Non-alcoholic steatohepatitis (NASH) or non-alcoholic fatty
viral infection in its etiology. Instead, a combination of some liver disease (NAFLD) is a from of hepatitis resembling
common toxic effects and inherited abnormality of copper alcoholic liver disease but seen in nondrinkers of alcohol.
metabolism has been suggested. Death occurs due to hepatic The condition is seen more commonly in affluent western
failure within a year of diagnosis. socieities, has a strong association with obesity,
dyslipidaemia and type 2 diabetes mellitus. It is seen in
MORPHOLOGIC FEATURES. Five histologic types of youger patients with equal gender prevalence. Pateints are CHAPTER 21
ICC have been distinguished of which type II is the most generally asymptomatic and are diagnosed by routine
common. This form is characterised by the following biochemical tests.
features: MORPHOLOGIC FEATURES. The condition is a form of
i) Liver cell injury ranging from ballooning degeneration chronic hepatitis after known causes have been excluded.
to significant damage to hepatocytes. About 10-30% cases of NASH progress to increased fibrosis
ii) Prominent Mallory bodies in some hepatocytes without and develop cirrhosis.
fatty change.
iii) Neutrophilic and sometimes alongwith lymphocytic Miscellaneous Forms of Cirrhosis
infiltrate. In addition to the various types of cirrhosis just described, a
iv) Creeping pericellular fibrosis which may eventually few other uncommon types associated with different diseases
lead to fine micro-macro-nodular cirrhosis. are sometimes distinguished. These include the following:
v) There is significant deposition of copper and copper- 1. Metabolic disorders e.g. in galactosaemia, hereditary
associated proteins in hepatocytes, often more than what fructose intolerance, glycogen storage diseases.
is seen in Wilson’s disease. 2. Infectious diseases e.g. in brucellosis, schistosomiasis, The Liver, Biliary Tract and Exocrine Pancreas
Thus, the picture resembles acute alcoholic hepatitis syphilis (hepar lobatum) and toxoplasma infection.
but without the fatty change and with greatly impaired 3. Gastrointestinal disorders e.g. in inflammatory bowel
regeneration. There is marked increase in hepatic copper disease, cystic fibrosis of the pancreas and intestinal bypass
since the milk consumed by such infants is often boiled surgery for obesity.
and stored in copper vessels in India. The condition has 4. Infiltrative diseases e.g. in sarcoidosis.
to be distinguished from Wilson’s disease.

Cryptogenic Cirrhosis
Cirrhosis in Autoimmune Hepatitis
Finally, when all the known etiologic types of cirrhosis have
Autoimmune hepatitis (also called lupoid hepatitis) is a form been excluded, there remain patients with cirrhosis in whom
of chronic hepatitis characterised by continued hepatocellular the cause is unknown. These cases are grouped under a
injury, inflammation and fibrosis which may progress to
cirrhosis. The condition may run a variable natural history waste-basket diagnosis of cryptogenic cirrhosis (crypto =
concealed).
rangingfrom indolent to severe rapid course. This form of
hepatitis has prominent autoimmune etiology is supported NON-CIRRHOTIC PORTAL FIBROSIS
by immunologic abnormalities and a few other characteristic
diagnostic criteria as under: Non-cirrhotic portal fibrosis (NCPF) is a group of congenital
1. Female gender predisposition. and acquired diseases in which there is localised or