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Ocular manifestations of monoclonal copper-binding immunoglobulin
Sejal Shah1, Edgar M Espana2, Curtis E Margo2
1Department of Ophthalmology, University of South Florida Morsani College of Medicine, Tampa, Florida, USA.
Insights
This study details a rare condition where copper accumulates in the eye due to a specific antibody. This copper buildup, linked to immunoglobulin overproduction, affects only ocular tissues.
Area of Science:
- Ophthalmology
- Immunology
- Toxicology
Background:
- A unique condition characterized by copper accumulation in the Descemet membrane and lens capsule.
- This phenomenon is linked to a circulating monoclonal antibody with high affinity for copper.
- The overproduction of monoclonal immunoglobulin may stem from multiple myeloma or benign monoclonal gammopathy.
Observation:
- A 46-year-old woman presented with clinical and pathological findings consistent with this rare disorder.
- Dense copper deposits were observed in the Descemet membrane and lens capsule.
- Elevated serum copper levels were noted, but without adverse effects on other body tissues.
Findings:
- The characteristic feature is ocular copper deposition driven by a specific monoclonal antibody.
- The condition is associated with monoclonal immunoglobulin overproduction.
- Despite significant copper exposure, systemic toxicity is absent, suggesting localized ocular effects.
Implications:
- This case highlights a specific mechanism of metal accumulation in ocular tissues.
- Understanding this antibody-copper interaction is crucial for diagnosing and managing related gammopathies.
- Further research may reveal targeted therapeutic strategies for this unique copper overload disorder.
Abstract:
The dense accumulation of copper in Descemet membrane and lens capsule is the characteristic manifestation of a circulating monoclonal antibody with strong affinity for copper. The overproduction of this monoclonal immunoglobulin may be associated with either multiple myeloma or a benign monoclonal gammopathy. Despite prolonged exposure to elevated serum copper, no other tissues in the body are adversely affected by this redox metal. We describe the clinical and pathological findings in a 46-year-old woman with this disorder.
