Who Needs a Liver Transplant?
Here are some of the most common reasons for liver transplantation in the BIDMC Transplant Institute.
Chronic Viral Hepatitis, B, C, D
Hepatitis can result from viral infection of the liver cells, causing inflammation, swelling, cell death and ultimately lead to liver destruction or scarring. There are many different types of viral hepatitis - A, B, C, D, and E - that specifically infect the liver. Some other viral infections that affect the entire body can also cause liver inflammation, such as herpes infection, cytomegalovirus, and Epstein Barr Virus, as part of their disease process. Hepatitis C virus, HCV, is the leading indication for liver transplants in the United States. Hepatitis B (and D) also cause chronic liver disease. Hepatitis B, HBV, is the leading indication for liver cancer worldwide.
This type of hepatitis is not caused by a viral infection. Rather, it is a condition where the body's immune system is unable to "recognize" its own liver tissue, and begins to cause liver cell injury that leads to inflammation and scarring. This scarring can ultimately lead to liver cirrhosis and end-stage liver disease.
The toxic effects of excessive alcohol can cause Laennec's Cirrhosis; that is, cirrhosis from chronic alcohol abuse.
Nonalcoholic Fatty Liver Disease (NAFLD)
Fat accumulation in the liver cells can occur for a number of reasons. Common causes of fat accumulation include excessive alcohol use; however, there is a recently recognized condition where fat accumulates in the livers of patients who do not drink alcohol. This is more common, but not restricted to, patients with diabetes and obesity. Rarely this condition can cause progressive inflammation and liver cell destruction resulting in scarring of the liver, a condition called Nonalcoholic Steato-Hepatitis (NASH). NASH is now becoming a leading cause of liver cirrhosis.
Sometimes people can develop cirrhosis or scarring that destroys the liver for no apparent reason, without ever having hepatitis or a history of substance abuse. This condition is called cryptogenic cirrhosis.
Cirrhosis or liver scarring, due to blockage in the bile duct, is called biliary cirrhosis. The liver produces bile, which flows through the bile duct to the gallbladder, where it is stored and then released into the small intestine to help digest fats. In biliary cirrhosis, the bile ducts become blocked, either from gallstones or possibly from an autoimmune reaction (when the body's own immune system attacks the tissue). With the bile ducts blocked, bile pools in the liver, causing swelling, cell death and loss of function.
Primary Sclerosing Cholangitis (PSC)
PSC is a condition in which the bile ducts become progressively damaged by inflammation, resulting in scarring, narrowing and obstruction to the flow of bile. This obstruction leads to jaundice (a yellowing of the eyes and skin), itch and fat malabsorption. The damage to the bile ducts can result in cirrhosis. There is a strong association between PSC and inflammatory bowel disease.
In this inherited and rare condition, bile ducts in the liver become dilated, resulting in bile duct cysts or sacks. These cystic dilatations may cause infection, gallstones, and obstruction. There is sometimes an association with congenital (present at birth) hepatic fibrosis (a scarring of the liver that can cause some of the same problems as cirrhosis).
Most likely a congenital disorder, a choledochal cyst forms because of a structurally flawed bile duct. Bile collects in the abnormal duct and forms a cyst, or sack, which blocks the bile from reaching the intestine. Instead of passing through to the intestine, the bile backs up into the liver, and this can cause infection, irritation and pain. Often undetected for years, the condition can lead to cirrhosis of the liver (scarring) and cancer.
Acute Liver Failure
People can suffer acute liver failure, with permanent loss of function, from viral hepatitis, fatty liver from pregnancy, and misuse of acetaminophen, a common pain reliever. People who exceed the recommended dose of acetaminophen - better known as the pain reliever Tylenol but contained in other over-the-counter medications, too - can suffer serious and permanent liver damage requiring a transplant. The liver metabolizes (changes into energy and nutrients) or eliminates most medications, like acetaminophen. Exceeding the recommended dose can disrupt liver function, particularly in a person who already has some type of liver disease.
For some people, exposure to pesticides, herbicides, lead, asbestos, radon and other hazardous environmental, occupational or household pollutants can lead to serious and potentially life-threatening liver disease. Some people are more susceptible (likely to be affected) than others.
Surgeons in the Transplant Institute perform liver transplantation for selected primary liver tumors (from cancer that begins in the liver) and for selected metastatic liver tumors (cancer that has spread to the liver from other organs). Selected tumors include:
- Neuroendocrine Tumors
These are tumors that grow from neuroendocrine cells in the gastrointestinal (GI or digestive) tract. Neuroendocrine cells have characteristics that are similar to both endocrine (hormone-producing) cells and nerve cells. If tumors in these cells metastasize (spread to) the liver, transplantation may be a treatment option.
- Hepatocellular Carcinoma
The most common type of liver cancer in adults, this cancer begins in the hepatocytes or liver cells. It may start as one single, growing tumor that eventually spreads to other areas of the liver, or it can start in many different parts of the liver at the same time. Selection for liver transplantation is based on the number and size of tumors, using either the Milan criteria or criteria developed by University of California, San Francisco (UCSF). According to the Milan criteria, patients appropriate for transplant should have either one tumor, 5 centimeters or less, or no more than 3 tumors, none larger in diameter than 3 centimeters. The UCSF criteria are slightly expanded to include one tumor, 6.5 centimeters or less, or no more than 3 tumors, none larger in diameter than 4.5 centimeters, with total tumor diameter not to exceed 8 centimeters. The BIDMC Transplant Institute uses the Milan criteria in most cases and the UCSF criteria in selected cases.
This type of vascular cancer targets blood vessels within soft tissue, liver, lung and other areas. Rare and slow growing, these tumors can also be benign.
- Slow Growing Metastatic Gastrointestinal Stromal Tumor (GIST)
A particular type of slow-growing tumor of the gastrointestinal (GI or digestive) tract, these tumors start in the special cells that line the wall of the GI tract.
Metabolic and Genetic Disorders
Patients with certain inherited diseases may benefit from a liver transplant. These diseases can include:
- Alpha 1 Antitrypsin Deficiency
People with this genetic disorder are missing a protective liver protein that defends against certain destructive enzymes. Without this protein, people may develop liver disease.
- Cystic Fibrosis
A genetic disease of the sweat and mucus glands that can seriously affect the liver and other organs.
- Familial Hypercholesterolemia
A genetic disorder that causes a dangerous buildup of cholesterol.
A genetic disorder that causes a dangerous buildup of iron levels within the body. Iron is deposited in the liver resulting in cirrhosis. Iron can also be deposited in the heart, pancreas, joints and pituitary gland.
- Wilson's Disease
A genetic disorder that causes a dangerous buildup of copper levels within the body.
- Glycogen Storage Disease
An inherited metabolic disorder, which interrupts how the body breaks down and stores nutrients for body fuel and energy. A defective or missing enzyme makes it impossible for the body to metabolize and store glycogen (or sugar). Glycogen is stored principally in the liver and muscles.
Patients with certain vascular diseases, such as Budd-Chiari Syndrome (BCS), may benefit from a liver transplant. BCS is clotting in the veins in the liver, which can lead to complications from deteriorating liver function. Patients with certain vascular diseases, such as Budd-Chiari Syndrome (BCS), may benefit from a liver transplant. BCS is clotting in the veins in the liver, which can lead to complications from deteriorating liver function.
A number of miscellaneous disorders and illnesses can cause liver failure, such as adult polycystic disease (multiple cysts or sacs within the liver); Alagille's syndrome (too few bile ducts in the liver); amyloidosis (abnormal deposits of the amyloid protein in liver tissue) and sarcoidosis (small lumps of cells in the liver).
Trauma to the liver, for example, injury from blunt force or other accidents, can lead to liver failure and ultimately a liver transplant.