Acquired monoclonal Ig light chain-associated Fanconi syndrome
Acquired monoclonal Ig light chain-associated Fanconi syndrome
Definition
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.
Also known as acquired Fanconi syndrome secondary to monoclonal gammopathy, acquired monoclonal immunoglobulin light chain-associated Fanconi syndrome — per MONDO