Ceruloplasmin

Serum (preferred) or plasma

1 mL

0.2 mL (Note: This volume does not allow for repeat testing.)

Red-top tube, gel-barrier tube, or green-top (heparin) tube

Decreased in most instances of Wilson's disease (hepatolenticular degeneration); hence, ceruloplasmin is used in evaluation of chronic active hepatitis, cirrhosis, and other liver disease. In Wilson's disease, there is decreased ability to incorporate copper into apoceruloplasmin. As a result, free copper levels in plasma and in tissue, especially liver and brain, are greatly increased.

Should be considered in cases of central nervous system disease of obscure etiology. Neurological symptoms include problems of coördination.

Ceruloplasmin is low in Menkes kinky hair syndrome (In Menkes syndrome the defect is secondary to poor absorption and utilization of dietary copper.) and with protein loss such as the nephrotic syndromes, malabsorption, and with some cases of advanced liver disease in which decreases of serum proteins have occurred.

Ceruloplasmin is high in a variety of neoplastic and inflammatory states, since it behaves as an acute phase reactant, although levels rise more slowly than “acute phase reactants.” Increases are described with carcinomas, leukemias, Hodgkin's disease, primary biliary cirrhosis, and with SLE and rheumatoid arthritis. High levels occur in pregnancy, with estrogens, and with oral contraceptive use when the agent contains estrogen as well as progesterone. Increased with copper intoxication.

A normal ceruloplasmin does not rule out Wilson's disease. Serum copper should be measured in addition.

Immunologic

See table.

Ceruloplasmin is an α₂-globulin containing copper. About 70% or more of total serum copper is associated with ceruloplasmin, 7% with a high MW protein, transcuprein, 19% with albumin, and 2% with amino acids.¹

Laboratory parameters of Wilson's disease include decreased serum ceruloplasmin, decreased serum copper concentration, increased 24-hour urine copper excretion, increased liver copper concentration, and abnormal liver function studies. Demonstration of failure to incorporate radiolabeled copper into ceruloplasmin is the definitive test for Wilson's disease. Liver and CNS manifestations of Wilson's disease need not both be present. Kayser-Fleischer rings are extremely helpful findings.

Excessive therapeutic zinc may lead to block of intestinal absorption of copper and a copper deficiency syndrome characterized by hypochromic microcytic anemia with leukopenia/neutropenia and zero level of ceruloplasmin. A prolonged period of time may be required to eliminate the excess zinc, overcome the block of intestinal copper absorption and obtain increase in serum copper and ceruloplasmin levels.²