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Kidney non-tumor

Associated with systemic conditions

Light chain deposition disease


Reviewers: Nikhil Sangle, M.D. (see Reviewers page)
Revised: 10 December 2014, last major update November 2012
Copyright: (c) 2003-2014, PathologyOutlines.com, Inc.

General
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● Monoclonal gammopathy characterized by overproduction and deposition of nonamyloid immunoglobulin light chains in various organs.(eMedicine)
● 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 Fanconiís 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
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● 41 year old man with no clinical plasma cell dyscrasia (Ultrastruct Pathol 2012;36:134)
● 49 year old woman with biopsy proven resolution after autologous stem cell transplantation.(Nephrol Dial Transplant 2010;25:2020)
● 53 year old woman with recurrent light chain deposition disease post-renal transplantat (Clin Transplant 2012;26 Suppl 24:64)
● 66 year old man presenting with severe jaundice (Oman Med J 2012;27:56)
● Middle-aged woman with myeloma and type II diabetes (Arch Pathol Lab Med 1983;107:319)

Treatment
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● Chemotherapy with bortezomib, a proteasome inhibitor (Med Oncol 2012;29:1197), thalidomide (Int J Hematol 2011;93:673); also autologous stem cell transplantation (Int J Lab Hematol 2012;34:347, J Nephrol 2011;24:246)

Micro description
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● 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

Micro images
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Various images


Light chain deposits along GBM


Mesangial nodular sclerosis and tubular injury on Silver stain


Mesangial nodular sclerosis on PAS


Mesangial nodular sclerosis on Silver stain


Mesangial nodular sclerosis on Trichrome stain


Negative staining for lambda light chain on IF


Positive GBM and TBM staining for kappa light chain on IF

Immunofluorescence
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● 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
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● Congo red, thioflavin T and amyloid P protein

Electron microscopy description
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● 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
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Light chain deposits along GBM


Light chain deposits along TBM

Differential diagnosis
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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

End of Kidney non-tumor > Associated with systemic conditions > Light chain deposition disease

Ref Updated: 7/31/12


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