AI Article Synopsis
- Light-chain deposition disease (LCDD) is a rare systemic condition that involves abnormal light-chain deposits in various organs, leading to potential organ failure, often associated with Bence-Jones type monoclonal gammopathy.
- A case study of a 65-year-old man with end-stage renal disease revealed he had cardiac LCDD instead of the initially suspected dialysis-associated cardiomyopathy, despite negative tests for cardiac amyloidosis.
- There is a need for greater awareness among clinicians about the risk of cardiac LCDD, particularly in patients with heart failure and renal issues, as it may go undetected unless thoroughly investigated.
Article Abstract
Background: Light-chain deposition disease (LCDD) is a systemic disorder characterized by non-amyloidotic light-chain deposition in various organs with Bence-Jones type monoclonal gammopathy. Although known as monoclonal gammopathy of renal significance, it may involve interstitial tissue of various organs, and in rare cases, proceeds to organ failure. We present a case of cardiac LCDD in a patient initially suspected of dialysis-associated cardiomyopathy.
Case Summary: A 65-year-old man with end-stage renal disease requiring haemodialysis presented with fatigue, anorexia, and shortness of breath. He had a history of recurrent congestive heart failure and Bence-Jones type monoclonal gammopathy. A cardiac biopsy performed for suspected light-chain cardiac amyloidosis was negative for diagnostic Congo-red stain, however, paraffin immunofluorescence examination for light-chain suggested diagnosis of cardiac LCDD.
Discussion: Cardiac LCDD may go undetected leading to heart failure due to lack of clinical awareness and insufficient pathological investigation. In heart failure cases with Bence-Jones type monoclonal gammopathy, clinicians should consider not only amyloidosis but also interstitial light-chain deposition. In addition, in patients with chronic kidney disease of unknown cause, investigation is recommended to rule out cardiac light-chain deposition disease concomitant with renal LCDD. Although LCDD is relatively rare it occasionally affects multiple organs; therefore, it would be better to describe it as a monoclonal gammopathy of clinical significance rather than one of renal significance.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9969531 | PMC |
| http://dx.doi.org/10.1093/ehjcr/ytad049 | DOI Listing |
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