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Coexistence of amyloidosis and light chain deposition disease in the heart

Yoshihiro Sobue1, Genzou Takemura2, Shunji Kawamura3

  • 1Department of Cardiovascular Medicine, Gifu Heart Center, Gifu, Japan.

Cardiovascular Pathology : the Official Journal of the Society for Cardiovascular Pathology
|December 2, 2020
PubMed

Insights

This case report details the rare coexistence of cardiac amyloid light-chain (AL) amyloidosis and light chain deposition disease (LCDD). Electron microscopy aids in diagnosing latent LCDD alongside cardiac amyloidosis.

Area of Science:

  • Cardiology
  • Nephrology
  • Hematology

Background:

  • Cardiac amyloid light-chain (AL) amyloidosis and light chain deposition disease (LCDD) share similar pathophysiologies stemming from plasma cell dyscrasias.
  • Reports on the coexistence of cardiac AL amyloidosis and LCDD are infrequent, highlighting a gap in clinical understanding and diagnosis.

Observation:

  • A 59-year-old male presented with exertional dyspnea, hypotension, and a 4-year history of renal dysfunction requiring hemodialysis.
  • Cardiac evaluation revealed severe systolic and diastolic dysfunction with chamber dilatation and granular sparkling, but not left ventricular hypertrophy.
  • Endomyocardial biopsy showed Congo red-positive deposits, indicative of cardiac amyloidosis, while electron microscopy revealed amorphous nonfibrillar deposits consistent with LCDD.

Findings:

  • The patient exhibited extremely high plasma-free light chain kappa (κ) levels with a κ/λ ratio of 1,919.
  • Light microscopy confirmed cardiac amyloidosis, while electron microscopy identified nonfibrillar light chain deposits, leading to a diagnosis of LCDD.
  • The study confirmed the coexistence of cardiac amyloidosis and LCDD in this patient.

Implications:

  • Electron microscopic assessment, in conjunction with Congo red staining, may be crucial for diagnosing subclinical or latent LCDD in patients with suspected cardiac light-chain amyloidosis.
  • This case underscores the importance of comprehensive diagnostic approaches to identify coexisting plasma cell dyscrasias affecting the heart and kidneys.
  • Further research into the shared mechanisms and clinical implications of coexisting cardiac AL amyloidosis and LCDD is warranted.

Keywords:
AL amyloidosisCardiomyopathycardiac amyloidosiselectron microscopyendomyocardial biopsylight chain deposition diseaseultrastructure

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