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light-chain deposition disease; Light chain nephropathy
Systemic disorder.
Etiology:
- overproduction & extracellular deposition of monoclonal light chains (multiple myeloma)
Pathology:
1) eosinophilic glomerular nodules
2) NOT congo-red positive
3) granular rather than fibrillar
4) a single class of light chains on immunofluorescent staining (80% kappa) found along the glomerular & tubular basement membranes
5) intratubular obstruction with light chains resulting in acute tubular injury
Clinical manifestations:
1) hypertension
2) orthostatic hypotension
3) renal insufficiency
Laboratory:
1) proteinuria
- serum protein electrophoresis
- generally no 'large' M spike
- urine protein electrophoresis
- light chains in urine
- urine dipstick (detects albumin) may suggests minimal proteinuria
2) renal biopsy with immunofluorescent staining show amyloid deposits
3) increased INR due to acquired factor X deficiency
- coagulation factor X inhibitor in plasma
Management:
- chemotherapy similar to renal amyloidosis
Related
myeloma cast nephropathy
renal amyloidosis; amyloid nephropathy
General
kidney disease; renal disease
References
- Medical Knowledge Self Assessment Program (MKSAP) 11, 18.
American College of Physicians, Philadelphia 1998, 2018