Liver Complications and Sickle Cell Disease

Sickle cell disease (SCD) or its treatments cause liver problems for many people. Sickling of blood cells in the liver can block blood flow and cause acute complications.

Blood transfusions also increase the risk for liver damage from iron overload and hepatitis. If you experience any symptoms of liver damage, call your doctor. They can determine the cause of the problem and advise you on treatment and lifestyle changes to prevent further damage.

What liver problems do people with sickle cell disease experience?

The liver is responsible for producing substances that digest food and eliminate toxins. A few different liver complications can occur for people with SCD. They may be due to the disease, to treatments, or both. The term sickle cell hepatopathy describes the overlapping causes of liver disease in people with SCD.1

People with SCD can experience an acute sickle hepatic crisis. This is an acute pain crisis that affects the liver and usually subsides with standard pain treatment. A more serious version is called intrahepatic cholestasis. This happens when the flow of bile from the liver is blocked, often because of gallstones. Repeated episodes can lead to chronic problems and liver failure.2,3

Another acute complication called hepatic sequestration crisis is rarer. This happens when red blood cells get trapped in the liver and cause it to enlarge. It can lead to anemia because there are not enough red blood cells circulating in the blood.4

Blood transfusions also increase the risk of liver damage for people with SCD. Iron overload from multiple transfusions can lead to severe liver damage, such as fibrosis or cirrhosis. Blood transfusions very rarely can cause hepatitis, which can progress to cirrhosis.5-7

Liver problems are usually diagnosed with blood and imaging tests. Doctors may analyze the levels of certain enzymes to determine liver function. They may also perform MRI and ultrasound tests to look at iron levels, gallstones, or other signs of liver damage.7

What causes liver problems?

The major risk factor is receiving multiple blood transfusions. This is linked to hepatitis infection (rarely) and high iron levels, which can both cause liver damage. However, SCD can cause liver problems in many other ways. Liver problems are most common in people with sickle cell anemia (HbSS).1

Sickle cells tend to get stuck in blood vessels and block blood flow. When this happens in the liver, liver cells cannot get enough oxygen. This can cause liver cell death and prevent the liver from working properly.8

Sickle cells also burst easily because they are unstable. When they burst, they release hemoglobin into the bloodstream. Hemoglobin gets broken down to a substance called bilirubin. When too many sickle cells burst, too much bilirubin builds up in the body.8

A high level of bilirubin (hyperbilirubinemia) is very common in people with SCD. This can form gallstones in the gallbladder, which can lead to liver problems. Very high bilirubin levels are seen in people with intrahepatic cholestasis.7

Symptoms

The symptoms for each liver condition may be slightly different. Common symptoms of acute liver episodes and liver damage include:7,9

  • Acute pain in the upper right part of the abdomen
  • Nausea
  • Low-grade fever
  • Enlarged liver
  • Jaundice
  • Abnormal urine and stool color

Contact your doctor if you show any of these symptoms. Many are also symptoms of other complications of SCD. Your doctor can perform tests to determine if your liver is functioning properly.

What are treatment and prevention options?

There are no medicines that reduce liver problems for people with SCD. Hydroxyurea may be used to reduce the need for blood transfusions and prevent iron overload. However, it does not affect the frequency of acute hepatic episodes.7,10

Some ways to prevent liver damage involve lifestyle changes, such as:9

  • Drinking minimal alcohol
  • Using a condom during sex
  • Avoiding sharing needles and contacting other people’s body fluids
  • Getting vaccinated for hepatitis
  • Maintaining a healthy weight
  • Making sure your food is clean and safe

Treatment of liver problems depends on the specific complication. Acute sickle hepatic crises are usually treated with hydration and pain medications. More serious liver problems may be treated with a blood transfusion.7

The risk for iron overload can be reduced by performing blood transfusions only when necessary and when other medications do not work. When chronic blood transfusions are necessary, exchange transfusions are safer than simple transfusions.11

For people receiving regular blood transfusions, iron chelation therapy may reduce iron accumulation in the liver. These medications bind iron and help remove them from the body. Some examples of iron chelating medications are deferoxamine, deferasirox, and deferiprone.11

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Written by: Matthew Zajac | Last reviewed: January 2021