Ceruloplasmin

At a Glance

Why Get Tested?

To measure the amount of ceruloplasmin in your blood; to help diagnose Wilson disease; sometimes to help identify conditions associated with copper deficiencies

When To Get Tested?

When you have jaundice, fatigue, abdominal pain, behavioral changes, tremors, or other symptoms that a healthcare practitioner thinks may be due to Wilson disease or, rarely, to copper deficiency; periodically when monitoring is recommended

Sample Required?

A blood sample drawn from a vein

Test Preparation Needed?

None

Looking For Test Results?

Looking For Reference Ranges?

You may be able to find your test results on your laboratory’s website or patient portal. However, you are currently at Testing.com. You may have been directed here by your lab’s website in order to provide you with background information about the test(s) you had performed. You will need to return to your lab’s website or portal, or contact your healthcare practitioner in order to obtain your test results.

Testing.com is an award-winning patient education website offering information on laboratory tests. The content on the site, which has been reviewed by laboratory scientists and other medical professionals, provides general explanations of what results might mean for each test listed on the site, such as what a high or low value might suggest to your healthcare practitioner about your health or medical condition.

The reference ranges for your tests can be found on your laboratory report. They are typically found to the right of your results.

If you do not have your lab report, consult your healthcare provider or the laboratory that performed the test(s) to obtain the reference range.

Laboratory test results are not meaningful by themselves. Their meaning comes from comparison to reference ranges. Reference ranges are the values expected for a healthy person. They are sometimes called “normal” values. By comparing your test results with reference values, you and your healthcare provider can see if any of your test results fall outside the range of expected values. Values that are outside expected ranges can provide clues to help identify possible conditions or diseases.

While accuracy of laboratory testing has significantly evolved over the past few decades, some lab-to-lab variability can occur due to differences in testing equipment, chemical reagents, and techniques. This is a reason why so few reference ranges are provided on this site. It is important to know that you must use the range supplied by the laboratory that performed your test to evaluate whether your results are “within normal limits.”

For more information, please read the article Reference Ranges and What They Mean.

What is being tested?

Ceruloplasmin is a copper-containing enzyme that plays a role in the body’s iron metabolism. This test measures the amount of ceruloplasmin in the blood.

Copper is an essential mineral that plays a role in the regulation of iron metabolism, formation of connective tissue, energy production within cells, and the function of the nervous system. It is absorbed from food and liquids by the intestines and then transported to the liver, where it is stored or used to produce a variety of enzymes.

The liver binds copper to a protein to produce ceruloplasmin and then releases it into the bloodstream. About 95% of the copper in the blood is bound to ceruloplasmin. Because of this, the ceruloplasmin test can be used along with one or more copper tests to help diagnose Wilson disease, an inherited disorder that can lead to excess storage of copper in the eyes, liver, brain, and other organs.

Common Questions

How is the test used?

Ceruloplasmin testing is used primarily, along with blood and/or urine copper tests, to help diagnose Wilson disease, a rare inherited disorder associated with excess storage of copper in the eyes, liver, brain, and other organs, and with decreased levels of ceruloplasmin.

Rarely, a ceruloplasmin test may be ordered with a copper test to help diagnose abnormal copper metabolism, copper deficiencies, or the rare inherited disorder Menkes kinky hair syndrome (see below).

When is it ordered?

A ceruloplasmin test may be ordered alone or along with blood and 24-hour urine copper tests when you have signs and symptoms that a healthcare practitioner suspects may be due to Wilson disease, such as:

Anemia
Nausea, abdominal pain
Jaundice
Fatigue
Behavioral changes
Tremors
Difficulty walking
Persistent involuntary muscle contractions that cause repetitive or twisting motions (dystonia)
Diarrhea and vomiting
Trouble swallowing or speaking
Dark brown rings around the iris of the eyes (Kayser-Fleischer rings)

Rarely, ceruloplasmin may also be ordered along with copper tests when a healthcare practitioner suspects that you have a copper deficiency. Symptoms of a copper deficiency include pale skin, osteoporosis, fatigue, and tingling in the hands and feet. Infants may be tested if they have signs of Menkes kinky hair syndrome, which include brittle, sparse or tangled hair, feeding problems, failure to grow, seizures, developmental delays, or low muscle tone.

Ceruloplasmin may be ordered periodically to monitor copper excess or deficiency and periodically to evaluate the effectiveness of treatment.

What does the test result mean?

Ceruloplasmin levels are not diagnostic of a specific condition and are usually evaluated along with copper tests.

Test results may include:

Test	Wilson Disease	Copper Toxicity	Menkes Disease (Kinky Hair Syndrome)	Copper Deficiency
Copper, blood	Low but may be normal	High	Low	Low
Copper, serum free	High	High	Low	Low
Ceruloplasmin	Low but may be normal	High	Low	Low
Copper, urine	Very high	High	Low	Low
Copper, liver/hepatic*	Positive but, depending on the site sampled, may be negative	High or normal	Low	Low

*Excess copper in the liver is often unevenly distributed and may not be detected in a sample.

Decreased ceruloplasmin and blood copper concentrations and increased urine copper levels may indicate Wilson disease.
Some people with Wilson disease who have neurological symptoms will have normal ceruloplasmin levels, as will many people with symptoms of liver damage, especially if they are acutely ill.
If ceruloplasmin and urine and/or blood copper concentrations are low, then the person tested may have a copper deficiency.
Anything that interferes with the supply of copper or with the body’s ability to metabolize copper has the potential to affect blood ceruloplasmin and copper concentrations.
Higher-than-normal ceruloplasmin levels could be a sign of a serious infection, heart disease, rheumatoid arthritis, leukemia or Hodgkin lymphoma.

Can I have the ceruloplasmin test done in my healthcare provider's office?

No. It is a specialized test that is not offered by every laboratory. Your blood sample may need to be sent to a reference laboratory.

Do I need to have a liver biopsy?

If Wilson disease is strongly suspected based upon blood, urine, and imaging test results, a liver biopsy may be performed to evaluate how much copper is in your liver and the extent of liver damage.

What is Menkes kinky hair syndrome?

Menkes kinky hair syndrome, also called copper transport disease, is a rare inherited disorder that causes a deficiency in copper. The syndrome is caused by mutations in the ATP7A gene located on the X chromosome. It is passed from parent to child in an X-linked recessive manner. This means that girls must inherit two copies of the mutated gene in order to be affected. Because boys only have one X chromosome, they can be affected if the mutation is present on the one X chromosome, making the condition much more common in males.

The mutation leads to uneven distribution of copper in the body. It may build up in tissues of the intestines and kidneys, for example, but may be deficient in areas such as the brain. Symptoms of the syndrome typically develop in infancy and many children die by age 3. Signs and symptoms include sparse, kinky hair, failure to grow at an expected rate and developmental delay, nervous system deterioration, weak muscle tone, and seizures. The incidence of this syndrome is about 1 in 100,000 infants.

Is there anything else I should know?

Ceruloplasmin may be increased in a variety of circumstances where the test is not used as a clinical tool. However, these conditions can affect interpretation of the test and the ability to recognize Wilson disease or copper deficiency. These may include the following:

Ceruloplasmin is an acute phase reactant. It is frequently elevated with inflammation, severe infection, tissue damage, and may be increased with some cancers.
It may be increased during pregnancy and with the use of estrogen, oral contraceptives, and medications such as carbamazepine, phenobarbital, and valproic acid.

Falsely low ceruloplasmin levels could occur in any condition that causes protein deficiency, such as nephrotic syndrome, malabsorption, loss of protein from the digestive tract (protein-losing enteropathy), and malnutrition.

View Sources

Sources Used in Current Review

Ceruloplasmin Test. U.S. National Library of Medicine MedlinePlus. Available online at https://medlineplus.gov/lab-tests/ceruloplasmin-test/. Accessed February 2020.

Kelly D, Crotty G, O’Mullane J, Stapleton M, Sweeney B, O’Sullivan SS. (January 2016) The Clinical Utility of a Low Serum Ceruloplasmin Measurement in the Diagnosis of Wilson Disease. Irish Medical Journal. Available online at https://www.ncbi.nlm.nih.gov/pubmed/26904791. Accessed February 2020.

Tapper EB, Rahni DO, Arnaout R, Lai M. (October 2013) The Overuse of Serum Ceruloplasmin Measurement. American Journal of Medicine. Available online at https://www.ncbi.nlm.nih.gov/pubmed/23953876. Accessed February 2020.

(Updated February 2019) Gilroy Richard. Wilson Disease Workup. Medscape. Available online at https://emedicine.medscape.com/article/183456-workup#c7. [Accessed February 2020]

(Updated January 2020) Wilson Disease. U.S. National Library of Medicine Genetics Home Reference. Available online at https://ghr.nlm.nih.gov/condition/wilson-disease. Accessed February 2020.

Sources Used in Previous Reviews

Clarke, W. and Dufour, D. R., Editors (2006). Contemporary Practice in Clinical Chemistry, AACC Press, Washington, DC. Christenson, R., Chapter 17, Proteins: Analysis and Interpretation in Serum, Urine, and Cerebrospinal Fluid. Pp. 197 – 210.

Cox, D. and Roberts, E. (2006 January 24). Wilson Disease. GeneReviews [On-line information]. Available online through http://www.genetests.org. Accessed on 7/17/07.

Thomas, Clayton L., Editor (1997). Taber’s Cyclopedic Medical Dictionary. F.A. Davis Company, Philadelphia, PA [18th Edition]. Pp. 353.

(2007 January). Wilson’s Disease Remains Difficult to Diagnose. Medscape from Reuters Health, from Gut 2007;56:115-120. [On-line information]. Available online at http://www.medscape.com/viewarticle/550386. Accessed on 7/27/07.

Das, S. and Ray, K. (2006 October 13). Wilson’s Disease: An Update. Medscape from Nature Clinical Practice Neurology [On-line information]. Available online at http://www.medscape.com/viewarticle/543866. Accessed on 7/27/07.

Van Voorhees, B. (2007 January 22). Ceruloplasmin. MedlinePlus Medical Encyclopedia [On-line information]. Available online at http://www.nlm.nih.gov/medlineplus/ency/article/003662.htm. Accessed on 7/27/07.

Wu, A. (2006). Tietz Clinical Guide to Laboratory Tests, Fourth Edition. Saunders Elsevier, St. Louis, Missouri. Pp. 230 – 233.

Dugdale, D. (Updated 2009 February 23). Ceruloplasmin. MedlinePlus Medical Encyclopedia [On-line information]. Available online at http://www.nlm.nih.gov/medlineplus/ency/article/003662.htm. Accessed November 2010.

(© 1995-2010). Unit Code 8364: Ceruloplasmin, Serum. Mayo Clinic, Mayo Medical Laboratories [On-line information]. Available online at http://www.mayomedicallaboratories.com/test-catalog/Overview/8364. Accessed November 2010.

McMillin, G. and Roberts, W. (Updated 2010 May). Wilson Disease. ARUP Consult [On-line information]. Available online at http://www.arupconsult.com/Topics/WilsonDz.html?client_ID=LTD#tabs=0. Accessed November 2010.

Mak, C. et. al. (2008 June 12). Diagnostic Accuracy of Serum Ceruloplasmin in Wilson Disease: Determination of Sensitivity and Specificity by ROC Curve Analysis among ATP7B-Genotyped Subjects. Clinical Chemistry. 2008;54:1356-1362 [On-line information]. Available online at http://www.clinchem.org/cgi/content/full/54/8/1356. Accessed November 2010.

Johnson, L. (Revised 2008 August). Copper. Merck Manual for Healthcare Professionals [On-line information]. Available online at http://www.merckmanuals.com/professional/sec01/ch005/ch005c.html?qt=wilson disease&alt=sh#sec01-ch005-ch005c-534. Accessed November 2010.

Tietz Textbook of Clinical Chemistry and Molecular Diagnostics. Burtis CA, Ashwood ER, Bruns DE, eds. St. Louis: Elsevier Saunders; 2006, Pp. 556 – 559.

(Updated 2014 July 23). Wilson Disease. National Institute of Diabetes and Digestive and Kidney Diseases [On-line information]. Available online at http://www.niddk.nih.gov/health-information/health-topics/digestive-diseases/wilson-disease/Pages/facts.aspx. Accessed December 2014.

Dugdale, D. (Updated 2013 February 2).Ceruloplasmin. MedlinePlus Medical Encyclopedia [On-line information]. Available online at http://www.nlm.nih.gov/medlineplus/ency/article/003662.htm. Accessed December 2014.

Strathmann, F. et. al. (Updated 2014 November). Wilson Disease. ARUP Consult [On-line information]. Available online at http://www.arupconsult.com/Topics/WilsonDz.html?client_ID=LTD. Accessed December 2014.

(© 1995–2014). Ceruloplasmin, Serum. Mayo Clinic Mayo Medical Laboratories [On-line information]. Available online at http://www.mayomedicallaboratories.com/test-catalog/Overview/8364. Accessed December 2014.

Johnson, L. (Revised 2013 April). Copper Deficiency and Toxicity. Merck Manual Professional Edition [On-line information]. Available online through http://www.merckmanuals.com. Accessed December 2014.

Gilroy, R. (Updated 2014 May 2). Wilson Disease. Medscape Drugs & Diseases [On-line information]. Available online at http://emedicine.medscape.com/article/183456-overview. Accessed December 2014.

Lorincz, M. (2012). Recognition and Treatment of Neurologic Wilson’s Disease. Medscape Multispecialty from Semin Neurol. 2012;32(5):538-543 [On-line information]. Available online at http://www.medscape.com/viewarticle/805129. Accessed December 2014.

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