What is the pathophysiology of light-chain deposition disease (LCDD)?

Updated: Sep 26, 2019
  • Author: Swapna Boppana, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Renal light-chain deposition disease (LCDD) is characterized by the presence of: nodular sclerosing glomerulonephritis on light microscopy; diffuse linear staining in the glomerular and tubular basement membrane by a single light chain on immunofluorescence; and nonfibrillar, powdery, electron-dense deposits in the basements on electron microscopy. [15] Mesangial nodularity within the glomerulus occurs from the increased deposition of extracellular matrix proteins mixed with kappa light chains. [15]

The necessary criterion to make the diagnosis of LCDD requires that all tissues to be stained for kappa and light chains and the tissue must exhibit kappa fixation along the tubular basement membrane. [16, 17, 18] The tubular deposits are present predominantly along the loops of Henle, distal tubules, and proximal tubules.

Amyloid light-chain (AL)–amyloidosis consists predominantly of lambda light chains, whereas kappa light chains are predominantly involved in LCDD. Electron microscopy is helpful in distinguishing between these lesions.


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