Light chain deposition disease
Franco Ferrario and Maria Pia Rastaldi
Dr Franco Ferrario
Renal Immunopathology Center
San Carlo Borromeo Hospital and Fondazione D'Amico per la ricerca sulle malattie renali
Monoclonal immunoglobulin deposition disease (MIDD) is a systemic disease, that can cause dysfunction in essentially any major organ. The diagnosis of MIDD includes systemic light chain deposition disease, light and heavy chain deposition disease, and heavy chain deposition disease. These diseases are defined by the composition of immunoglobulin chains demonstrated in deposits in the kidneys and other viscera.
Typically, by electron microscopy, a linear subendothelial electrondense deposition can be found, with the aspect of a dark line. Sometimes, instead, deposition has a more granular appearance.
By light microscopy, the glomerular lesions observed in LCDD can be extremely variable. Minimal glomerular alterations or only a mild mesangial expansion can be found. In these cases differential diagnosis has to be made from minimal change disease.
A mesangial and capillary wall segmental deposition of amorphic material is present in other cases.
By immunofluorescence a mesangio-parietal positivity of the light chain (k in this biopsy) allows the diagnosis.
Electrondense deposits can be observed not only at subendothelial but also at intramembranous level.
Light chain deposition can produce an important thickening of the basement membrane, giving rise to a picture similar to stage III MGN.
Nodular glomerulosclerosis is commonly found, and has to be differentiated from other nodular glomerulonephritis, such as idiopathic MPGN, diabetic glomerulosclerosis, and amyloidosis.
Huge nodules are often present.
Light chain deposition can involve the Bowman's capsule.
The same deposition can also cause tubular basement membrane thickening.
In these cases, tubular basement membranes show k or l chain positivity by immunofluorescence.
A huge light chain deposition is also commonly found in renal arterioles, with lumen narrowing.
Medium and large size arteries can also be involved.
Also by electron microscopy deposits can be found in the vessel walls.
|1.||Touchard G, Bridoux F, Goujon JM. Glomerulonephritis with organized immunoglobulin deposits. Nephrol Ther 2005 Dec; 1(6):355-64.|
|2.||Sirohi B, Powles R. Epidemiology and outcomes research for MGUS, myeloma and amyloidosis. Eur J Cancer 2006 Jul; 42(11):17671-83.|
|3.||Touchard G. Bridoux F, Goujon JM. Glomerulonephritis with organized immunoglobulin deposits. Nephrol Ther 2005 Dec; 1(6):355-64.|
|4.||Alpers CE, Tu WH, Hopper J Jr, Biava CG. Single light chain subclass (kappa chain) immunoglobulin deposition in glomerulonephritis. Hum Pathol 1985 Mar ; 16(3):294-304.|
|5.||Pozzi C, Locatelli F. Kidney and liver involvement in monoclonal light chain disorders. Semin Nephrol 2002 Jul; 22(4):319-30.|
|6.||Santostefano M, Zanchelli F, Zaccaria A, Poletti G, Fusaroli M. The ultrastructural basis of renal pathology in monoclonal gammopathies. H Nephrol 2005 Nov-Dec 18(6):659-75.|
|7.||Buxbaum J. Mechanisms of disease: monoclonal immunoglobulin deposition. Amyloidosis, light chain deposition disease, and light and heavy chain deposition disease. Hematol Oncol Clin North Am 1992 Apr; 6(2):323-46.|
|8.||Buxbaum J, Gallo G. Nonamyloidotic monoclonal immunoglobulin deposition disease. Light-chain, heavy-chain, and light- and heavy-chain deposition diseases. Hematol Oncol Clin North Am 1999 Dec; 13(6):1235-48.|