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Acquired monoclonal Ig light chain-associated Fanconi syndrome

Disease definition

A rare plasma cell dyscrasia 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.

ORPHA:91136

Classification level: Disorder
  • Synonym(s):
    • Acquired Fanconi syndrome secondary to monoclonal gammopathy
    • Acquired monoclonal immunoglobulin light chain-associated Fanconi syndrome
  • Prevalence: <1 / 1 000 000
  • Inheritance: Not applicable 
  • Age of onset: Adult, Elderly
  • ICD-10: E72.0
  • OMIM: -
  • UMLS: -
  • MeSH: -
  • GARD: -
  • MedDRA: -

Additional information

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