Acquired monoclonal Ig light chain-associated Fanconi syndrome

What is Acquired monoclonal Ig light chain-associated Fanconi syndrome?

A rare monoclonalgammopathy characterized by renal proximal tubule dysfunction secondary to monoclonal kappa light chain deposits in proximal tubular cells. Clinical presentation is with variable chronic kidney disease, low molecular weight proteinuria, aminoaciduria, hyperphosphaturia, uricosuria, bicarbonaturia, and non-diabetic glycosuria. Renal phosphate and urate wasting may cause hypophosphatemia and hypouricaemia.

Other condition names

  • Acquired Fanconi syndrome secondary to monoclonal gammopathy
  • Acquired monoclonal immunoglobulin light chain-associated Fanconi syndrome

Inheritance type

Not applicable


  • Worldwide: <1 in 1,000 000

Age of Onset

  • Adult
  • Elderly
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