Kidney non-tumor

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Kidney non-tumor

Associated with systemic conditions

Myeloma

Reviewers: Nikhil Sangle, M.D. (see Reviewers page)
Revised: 23 December 2012, last major update July 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● See also light chain deposition disease, Lymphoma and plasma cell neoplasms chapter
● Renal dysfunction / insufficiency occurs in 50% and renal disease is #2 cause of death in myeloma patients

Renal features
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● Various renal disorders (Arch Pathol Lab Med 2004;128:875, Am J Kidney Dis 2012;59:786):

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 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)
● Estimated GFR is calculated using Modification of Diet in Renal Disease (MDRD) Study equation, which has prognostic impact (Eur J Haematol 2012;88:159)

Case reports
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● 51 year old man presenting with rapidly progressive glomerulonephritis due to IgA myeloma (Nephron Extra 2011;1:69)

Treatment of renal disease
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● Treatment for underlying plasma cell neoplasm is discussed in Lymphoma and plasma cell neoplasms chapter
● Dialysis for acute renal failure (Nefrologia 2012;32:35, Ann Hematol 2012;91:729)

Micro description
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● 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)

Micro images
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Light chain cast nephropathy-various images

Plasma cell nodules

Left: thrombotic microangiopathy; right: abscess with fungal hyphae

Glomerular disease: various images

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

Tubular disease: various images

Various images

Immunofluorescence
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● Granular dense material along basement membrane

Electron microscopy description
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● Peripheral and mesangial kappa or lambda light chains

Differential diagnosis
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● Diabetic nodules
● Membranoproliferative glomerulonephritis

End of Kidney non-tumor > Associated with systemic conditions > Myeloma

Ref Updated: 7/31/12

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