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Updated: Feb 20, 2026

Genetic Analysis of Hereditary Transthyretin Ala97Ser Related Amyloidosis
Published on: June 9, 2018
Atypical case of AL amyloidosis with urinary erythrocyte casts
Orfeas Liangos1, Maike Buettner-Herold2, Markus Ketteler1
1Klinikum Coburg, III. Medizinische Klinik, Coburg.
Abstract:
We present the case of a 73-year-old man who developed an acute, severe febrile illness with multiorgan dysfunction, featuring renal failure, nephrotic-range proteinuria, microhematuria, and a skin rash. Numerous erythrocyte casts were found on urine microscopy. Typically, the finding of urinary erythrocyte casts indicates the presence of an underlying glomerular inflammatory disease. However, on renal biopsy, only amyloid light-chain (AL) amyloidosis and tubular injury were the predominant findings with no signs of glomerular or vascular inflammation. Photomicrographs of urinary sediment as well as renal biopsy histopathology of the presented case are shown. The unusual combination of findings, is then discussed in light of the existing literature on renal amyloidosis as well as erythrocyte casts in conditions other than glomerulonephritis.
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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