Kidney non-tumor - Light chain deposition disease

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Kidney non-tumor

Associated with systemic conditions

Light chain deposition disease

Reviewers: Nikhil Sangle, M.D. (see Reviewers page)
Revised: 21 November 2012, last major update November 2012
Copyright: (c) 2003-2012, 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 Fanconi’s 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

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)

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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