What does a blood test reading of hypercupremia indicate and how is it treated?

Elevated Blood Copper Level (Hypercupremia): Diagnosis and Management

A blood copper level of 67 indicates hypercupremia, which most commonly suggests Wilson's disease, but could also be associated with certain malignancies, particularly chronic lymphocytic leukemia with monoclonal gammopathy. Proper diagnosis and treatment are essential to prevent serious complications including liver damage, neurological deterioration, and corneal copper deposition.

Diagnostic Approach for Hypercupremia

Step 1: Initial Assessment

• Measure serum ceruloplasmin levels
  • Extremely low ceruloplasmin (<50 mg/L or <5 mg/dL) strongly suggests Wilson's disease 1
  • Normal or elevated ceruloplasmin with elevated copper suggests other causes of hypercupremia

Step 2: Calculate Non-Ceruloplasmin Bound Copper

• Non-ceruloplasmin bound copper = serum copper (μg/dL) - 3 × ceruloplasmin (mg/dL) 1
• Values >25 μg/dL (>250 μg/L) suggest pathological copper excess
• This calculation is more valuable than total copper alone

Step 3: Additional Testing

• 24-hour urinary copper excretion

  • 100 μg/24 hours (>1.6 μmol/24 hours) suggests Wilson's disease 1

• Complete blood count to assess for:
  • Anemia and neutropenia (may indicate copper toxicity)
  • Evidence of hematologic malignancy 2
• Ophthalmologic examination for:
  • Kayser-Fleischer rings (Wilson's disease)
  • Central brown-colored corneal opacity (monoclonal gammopathy-associated hypercupremia) 3, 4
• Serum protein electrophoresis if monoclonal gammopathy suspected 4

Treatment Algorithm Based on Etiology

For Wilson's Disease

1. First-line treatment: Chelation therapy

   • Trientine: 750-1500 mg/day in 2-3 divided doses 1
   • Take 1 hour before or 2 hours after meals 5
   • Monitor for potential side effects including iron deficiency 5

2. Alternative treatment: Zinc therapy

   • Zinc acetate: 50 mg three times daily 6
   • Acts by blocking intestinal absorption of copper
   • Take on an empty stomach, at least 1 hour apart from food or other medications 6
   • Less side effects than chelation therapy

3. Monitoring treatment efficacy

   • 24-hour urinary copper excretion:
     • 200-500 μg/day (3-8 μmol/day) for patients on chelation therapy 1
     • <75 μg/day (1.2 μmol/day) for patients on zinc therapy 1
   • Non-ceruloplasmin bound copper should normalize with effective treatment
   • Values <5 μg/dL (50 μg/L) suggest overtreatment 1

For Hypercupremia Associated with Malignancy

1. Treat the underlying malignancy 7, 4

   • Cytotoxic drugs for multiple myeloma or chronic lymphocytic leukemia
   • Consult with hematology/oncology

2. Consider limited chelation therapy 7

   • May have limited response without treating underlying condition

Special Considerations

Monitoring Schedule

• Initial phase of treatment: Monitor every 1-2 months
• Maintenance phase: Monitor at least twice yearly 1
• Laboratory monitoring should include:
  • Liver function tests
  • Complete blood count
  • Serum copper and ceruloplasmin
  • 24-hour urinary copper excretion
  • Non-ceruloplasmin bound copper calculation

Common Pitfalls to Avoid

1. Misdiagnosis: Not all hypercupremia is Wilson's disease; consider hematologic malignancies with monoclonal gammopathy 7, 3, 4

2. Overtreatment: Excessive chelation can lead to copper deficiency with:

   • Neutropenia
   • Anemia
   • Neurological symptoms 1, 2

3. Inadequate monitoring: Failure to regularly assess copper status can lead to:

   • Disease progression with inadequate treatment
   • Copper deficiency with excessive treatment

4. Drug interactions:

   • Trientine chelates iron; avoid coadministration 1
   • Zinc reduces absorption of other medications; maintain proper spacing 6

Early diagnosis and appropriate treatment of hypercupremia are critical to prevent irreversible neurological damage, liver disease, and other complications associated with copper toxicity.