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Systemic lambda light-chain deposition presenting with predominant cardiac involvement

M J Garton1, S Walton, S W Ewen

  • 1Department of Cardiology, Aberdeen Royal Infirmary, Foresterhill, UK.

Postgraduate Medical Journal
|July 1, 1993
PubMed
Summary
This summary is machine-generated.

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This study details a rare case of cardiac disease caused by non-amyloidotic light-chain deposition in an elderly woman with suspected Bence Jones myeloma. Postmortem analysis revealed lambda light-chain deposits in the heart and kidneys, contributing to heart failure and bradycardia.

Area of Science:

  • Cardiology
  • Nephrology
  • Hematology

Background:

  • Light-chain deposition disease (LCDD) is a rare condition often associated with plasma cell dyscrasias.
  • Cardiac involvement in LCDD can lead to heart failure and arrhythmias.
  • Non-amyloidotic light-chain deposits are distinct from amyloid fibrils and require specific diagnostic criteria.

Observation:

  • An 82-year-old woman with suspected Bence Jones myeloma presented with severe fluid retention and angina.
  • She required permanent cardiac pacing for symptomatic sinus bradycardia.
  • Autopsy revealed significant myocardial and renal deposits of lambda light-chains.

Findings:

  • The light-chain deposits were Congo Red negative, indicating they were non-amyloidotic.
  • Electron microscopy showed a non-fibrillar ultrastructure of the deposits.

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  • These findings suggest a rare cause of cardiac and renal pathology in this patient.

    • Implications:

    • This case highlights non-amyloidotic light-chain deposition as an uncommon but significant cause of cardiac disease.
    • It underscores the importance of considering light-chain deposition in patients with myeloma and cardiac symptoms.
    • Further research into the pathogenesis and treatment of non-amyloidotic LCDD is warranted.