Explanation

Ceruloplasmin: what is measured?

Ceruloplasmin is a protein made by the liver. It binds copper and carries it; most of the copper in the blood is attached to ceruloplasmin. Copper comes from food, and what the body does not need is removed by the liver through the bile. In Wilson's disease, this removal does not work properly: copper builds up in the liver, brain and eyes, among other places, and ceruloplasmin is then usually low. Ceruloplasmin is also an inflammatory protein, so the value actually rises with inflammation.

What can it detect?

Wilson's disease: a rare inherited disease in which copper builds up in the liver, brain and eyes (then usually low)
Copper deficiency due to malnutrition or poor absorption of nutrients (then low)
Protein loss through the kidneys or severe liver disease (then low)
Inflammation, pregnancy or use of the contraceptive pill (then raised)

Result

What does the result mean?

Ceruloplasmin too high

A raised ceruloplasmin usually has a temporary or harmless cause. The value rises with inflammation, infection and after an injury, and also during pregnancy and with use of the contraceptive pill or other products containing estrogen. According to the Dutch Society for Clinical Chemistry (NVKC), the value can also be higher with disorders of the bile ducts. A raised value does not mean that there is too much copper in the body.

Ceruloplasmin too low

A low ceruloplasmin may fit Wilson's disease, especially together with unexplained liver abnormalities in a young person, or with symptoms such as trembling, difficulty writing, speaking or swallowing, or changes in mood and behavior. More often, a low value has another cause: malnutrition, poor absorption of nutrients in the gut, protein loss through the kidneys or severe liver disease. A copper deficiency also lowers the value; this can develop, for example, after weight-loss stomach surgery or with long-term use of high doses of zinc. According to the Dutch Liver Patients Association (Nederlandse Leverpatiënten Vereniging), carriers of one abnormal Wilson gene, who do not become ill themselves, can also have a low value.

After an abnormal result

With a low ceruloplasmin, the GP looks at liver values, diet, possible protein loss through the kidneys and zinc use, and repeats the measurement if needed. If the result and the symptoms fit Wilson's disease, referral follows to a gastroenterologist or an expertise center for copper in 24-hour urine, an eye examination and DNA testing.

Reliability

How reliable is the test?

Ceruloplasmin alone cannot confirm or rule out Wilson's disease. According to the Dutch Society for Clinical Chemistry (NVKC), Wilson's disease is very unlikely with a normal value, but according to the Dutch Liver Patients Association (Nederlandse Leverpatiënten Vereniging), a normal or slightly raised value does not rule out the disease, partly because inflammation and hormone use raise the value. A low value can also be found in carriers and in other conditions. The diagnosis is made in hospital with a combination of ceruloplasmin, copper in 24-hour urine, an eye examination for a copper ring around the iris (Kayser-Fleischer ring) and DNA testing.

What can affect the result?

Inflammation, infection, pregnancy and products containing estrogen raise the value and can mask a low value.
Malnutrition, protein loss through the kidneys or gut and severe liver disease lower the value, regardless of Wilson's disease.
Measurement methods and reference values differ between laboratories.
A low value in a carrier of one abnormal gene does not mean that the person will get the disease.

Who it is for

Who is the test useful for?

Useful for

Useful with unexplained liver abnormalities or hepatitis at a young age, with unexplained symptoms such as trembling or difficulty writing, speaking or swallowing together with abnormal liver values, and in brothers, sisters or children of someone with Wilson's disease (then usually together with DNA testing). The test is also used to monitor treatment. In people without symptoms or family history, the chance of Wilson's disease is so small that a low result almost always has another cause.

How common is it?

Wilson's disease is rare: according to Erfelijkheid.nl, about 1 in 30,000 people have the disease, and according to the Dutch Liver Patients Association (Nederlandse Leverpatiënten Vereniging), 3 to 4 people in the Netherlands are diagnosed each year. About 1 in 100 people carry one abnormal gene and do not become ill themselves. The first symptoms usually start between the ages of 10 and 30.

Erfelijkheid.nl (Erfocentrum); Dutch Liver Patients Association (Nederlandse Leverpatiënten Vereniging); MDL Fonds

Drawbacks and risks

A slightly low value without symptoms usually has a cause other than Wilson's disease, but can lead to unnecessary worry and extensive follow-up tests.
A normal value can wrongly reassure you when you have symptoms that fit Wilson's disease.

Get tested

Ceruloplasmin: test panels

Ceruloplasmin is part of these test panels. You order online and have your blood drawn at a collection point or collect the sample at home.

Blood draw
Ceruloplasmin
Copper transport protein; useful for assessing copper disorders.
€ 35,00

Evidence

Sources

This explanation is based on Dutch guidelines and independent information. It is general information, not a diagnosis. Discuss an abnormal result with your GP.

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