Kidney nontumor / medical renal

Monoclonal gammopathy of renal significance (MGRS) / paraprotein-related kidney disease

Light chain deposition disease



Last author update: 1 December 2014
Last staff update: 20 July 2022

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PubMed Search: Light chain deposition disease kidney pathology

Nikhil Sangle, M.D.
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Cite this page: Sangle N. Light chain deposition disease. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/kidneylightchaindepositiondisease.html. Accessed February 6th, 2023.
Definition / general
  • Monoclonal gammopathy characterized by overproduction and deposition of nonamyloid immunoglobulin light chains in various organs.(eMedicine: Light-Chain Deposition Disease [Accessed 17 January 2018])
  • Uncommon; occasionally also heavy chains
  • See also myeloma
  • 80% male, usually older adults
  • 60% have associated myeloma or other lymphoplasmacytic disorder, although it may not become apparent until years later
  • Renal failure with heavy proteinuria; also Fanconi anemia with aminoaciduria, glucosuria and phosphaturia
  • Also cardiac, hepatic and neural damage and deposition in soft tissues and other organs of histiocytes and fibroblasts containing crystals (Am J Surg Pathol 1993;17:461)
  • May recur in renal transplants
  • Variable 5 year survival; ~ 70%, less if coexisting myeloma
Case reports
Treatment
Microscopic (histologic) description
  • Enlarged glomeruli with PAS+ material in thickened capillary walls and mesangial nodules
  • Occasional fibroepithelial crescents
  • Thickened tubular basement membranes with glassy (crystalline) appearance
  • Also crystals within histiocytes; weakly positive on silver stain
Microscopic (histologic) images

Contributed by NephroPath
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Light chain deposits along GBM

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Mesangial nodular
sclerosis and
tubular injury
on silver stain
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Mesangial nodular sclerosis on PAS

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Mesangial nodular sclerosis on silver stain

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Mesangial nodular sclerosis on trichrome stain



Images hosted on other servers:
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Various images

Immunofluorescence description
  • Granular deposits of kappa (80%) or lambda (20%) light chains (not both) along glomerular and tubular basement membranes, in mesangium, vessel walls and interstitium
Immunofluorescence images

Contributed by NephroPath
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Negative staining for lambda light chain on IF

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Positive GBM and
TBM staining
for kappa light
chain on IF

Negative stains
  • Congo red, thioflavin T and amyloid P protein
Electron microscopy description
  • Diffuse electron dense, finely granular material in glomerular basement membrane, mesangium, tubular and vascular basement membranes
  • Immunoelectron microscopy may be useful for diagnosis (Hum Pathol 2003;34:270)
Electron microscopy images

Contributed by NephroPath
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Light chain deposits along GBM

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Light chain deposits along TBM

Differential diagnosis
  • AL amyloidosis: fibrillar deposits, usually lambda light chains, Congo Red+, thioflavin T+, amyloid P protein+
  • Diabetes: severe arteriolar hyalinosis, fibrin caps, capsular drops, strongly positive with silver stain
  • Drug related crystals
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