light-chain deposition disease; Light chain nephropathy

Introduction

Systemic disorder.

Etiology

• overproduction & extracellular deposition of monoclonal light chains (multiple myeloma)

Pathology

• eosinophilic glomerular nodules
• NOT congo-red positive
• granular rather than fibrillar
• a single class of light chains on immunofluorescent staining (80% kappa) found along the glomerular & tubular basement membranes
• intratubular obstruction with light chains resulting in acute tubular injury

Clinical manifestations

• hypertension
• orthostatic hypotension
• renal insufficiency

Laboratory

• proteinuria
  • serum protein electrophoresis
    • generally no 'large' M spike
  • urine protein electrophoresis
    • light chains in urine
  • urine dipstick (detects albumin) may suggests minimal proteinuria
• renal biopsy with immunofluorescent staining show amyloid deposits
• increased INR due to acquired factor X deficiency
  • coagulation factor X inhibitor in plasma

Management

• chemotherapy similar to renal amyloidosis

More general terms

• kidney disease; renal disease

Additional terms

• myeloma cast nephropathy
• renal amyloidosis; amyloid nephropathy

Patient information

light chain deposition disease patient information