Description
Caeruloplasmin is a serum copper transport protein. Serum caeruloplasmin concentrations are low in patients with severe liver disease irrespective of aetiology. However, measurement of serum caerulopasmin, in conjunction with plasma and urinary copper levels are particularly useful in the diagnosis of Wilsons disease. Low values of caerulopasmin are also seen in malnutrition, malabsorption and nephrotic syndrome. Elevated values are seen in acute phase response associated with infections, malignancy or trauma. Particularly high values are seen in lymphoma and infection or obstruction of the biliary tract. Inflammation/infection may mask a deficiency. Anticonvulsants and oral contraceptives may also increase levels.
Indication
Diagnosis of Wilson’s disease.
Interpretation
95% patients with Wilson’s disease have low caeruloplasmin levels. Useful diagnostic criteria for Wilson’s disease are as follows:
- Serum caeruloplasmin < X
- Increased copper in liver biopsy (>250ug/g dry weight in the absence of signs of other cholestatic liver disease).
- Increase in urinary copper > 0.8umol/24hr.
- Increased non-caeruloplasmin bound copper.
- Kayser-Fleischer rings (present in 75% patients).
Sample
Serum Separator Tube (SST)
Assay details
Nephelometry
Restrictions
Increased levels of lipids (lipaemia), haemoglobin (haemiolysis), or the presence of icterus in the assay sample may affect the assay result.
Reference range
Female
- up to 4 months 0.15 – 0.56
- up to 4y 0.26 – 0.90
- up to 13y 0.25 to 0.46
- up to 40y 0.22 to 0.60
- up to 100y 0.25 to 0.60
Male
- up to 4 months 0.15 – 0.56
- up to 4y 0.26 – 0.90
- up to 13y 0.25 to 0.46
- up to 20y 0.15 to 0.37
- up to 100y 0.22 to 0.40
Assay range notes
Caeruloplasmin levels are slightly dependent on age and gender particularly in very young children (
Turnaround time
3 – 5 days
Analysing laboratory
Immunology JCUH