Atypical case of AL amyloidosis with urinary erythrocyte casts

Orfeas Liangos1, Maike Buettner-Herold2, Markus Ketteler1

  • 1Klinikum Coburg, III. Medizinische Klinik, Coburg.

Insights

This case study highlights amyloid light-chain amyloidosis presenting with erythrocyte casts, a finding usually linked to glomerulonephritis. Renal biopsy revealed amyloidosis, not inflammation, challenging typical diagnostic assumptions.

Area of Science:

  • Nephrology
  • Pathology
  • Internal Medicine

Background:

  • Erythrocyte casts in urinalysis typically signify glomerular inflammation, such as glomerulonephritis.
  • Amyloid light-chain (AL) amyloidosis is a plasma cell dyscrasia that can lead to systemic amyloid deposition.
  • Renal involvement in AL amyloidosis commonly presents with proteinuria and renal insufficiency.

Observation:

  • A 73-year-old male presented with acute febrile illness, multiorgan dysfunction, renal failure, nephrotic-range proteinuria, microhematuria, and a rash.
  • Urinary microscopy revealed numerous erythrocyte casts.
  • Renal biopsy demonstrated AL amyloidosis and tubular injury, notably lacking signs of glomerular or vascular inflammation.

Findings:

  • The presence of erythrocyte casts in this patient was not indicative of glomerulonephritis.
  • Renal biopsy confirmed AL amyloidosis as the primary pathology despite the urinary findings.
  • This case illustrates an atypical presentation of renal amyloidosis.

Implications:

  • Diagnostic criteria for renal diseases should consider atypical presentations of amyloidosis.
  • The association between erythrocyte casts and non-glomerular conditions like amyloidosis warrants further investigation.
  • Understanding these unusual findings can improve diagnostic accuracy and patient management for rare kidney diseases.

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