Kidney nontumor

Monoclonal gammopathy of renal significance (MGRS) / paraprotein-related kidney disease

Light chain cast nephropathy



Topic Completed: 3 February 2014

Minor changes: 13 September 2021

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PubMed Search: light chain cast nephropathy [title]

Nikhil Sangle, M.D.
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Cite this page: Sangle N. Light chain cast nephropathy. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/kidneylightchaincastnephropathy.html. Accessed December 8th, 2021.
Definition / general
Renal Features
    Bence Jones nephropathy
  • Also called myeloma cast nephropathy or myeloma kidney
  • Occurs in 30 - 40% at autopsy
  • Proteinuria consisting of light chains, which are normally filtered by glomeruli, reabsorbed and metabolized by proximal tubular cells
  • In myeloma, proximal tubular cells are overwhelmed and light chains reach distal nephron, where they are directly toxic to epithelial cells; Bence Jones proteins combine with urinary glycoprotein (Tamm-Horsfall) under acidic conditions to form tubular casts that obstruct tubules

  • AL amyloidosis (6 - 24% of myeloma patients)
  • Hypercalcemia, hyperuricemia, urinary tract obstruction, vascular disease
  • Light chain nephropathy: PAS+ mesangial deposits in glomeruli, lobular accentuation, mesangial hypercellularity
  • Also acute and chronic renal failure induced by dehydration, acute infection, acute tubular necrosis (acute tubulopathy), fungal infection, infarction, monoclonal cryoglobulins in glomeruli, neprhotic syndrome, nephrotoxic antibiotics, plasma cell tumor nodules, thrombotic microangiopathy, tubulointerstitial nephritis

  • Estimated GFR is calculated using Modification of Diet in Renal Disease (MDRD) Study equation, which has prognostic impact (Eur J Haematol 2012;88:159)
  • Note: urinary beta 2 microglobulinemia can simulate Bence-Jones proteins in electrophoresis (Arch Pathol Lab Med 2001;125:555)
Case reports
  • 51 year old man presenting with rapidly progressive glomerulonephritis due to IgA myeloma (Nephron Extra 2011;1:69)
Treatment of renal disease
Microscopic (histologic) description
  • Pink-blue amorphous masses fill and distend lumen, surrounded by giant cells and histiocytes; adjacent epithelium is necrotic
  • Casts and histiocytes may contain rhomboid or needle shaped crystals (light chains)
Microscopic (histologic) images

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Light chain cast with cellular reaction on silver stain



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Light chain cast nephropathy

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Glomerular disease: crescentic glomerulonephritis as presenting sign of IgA myeloma (rare)

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Various images

Immunofluorescence description
  • Granular dense material along basement membrane
Electron microscopy description
  • Peripheral and mesangial kappa or lambda light chains
Electron microscopy images

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Positive casts stain for lambda light chain on IF-2

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Kappa light chain negative casts on IF-2

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