Kidney nontumor
Associated with systemic conditions
Light chain deposition disease

Author: Nikhil Sangle, M.D. (see Authors page)

Revised: 23 March 2018, last major update December 2014

Copyright: (c) 2003-2018, PathologyOutlines.com, Inc.

PubMed Search: Light chain deposition disease [title] kidney pathology

Cite this page: Sangle, N. Light chain deposition disease. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/kidneylightchaindepositiondisease.html. Accessed October 18th, 2018.
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‚Äôs 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

Images hosted on PathOut server:
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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

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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

Images hosted on other servers:
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Various images

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

Images hosted on PathOut server:
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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