A patient with light chain disease may develop a renal Fanconi syndrome, which may be partial or complete.
Clinical features:
(1) light chain disease, usually with kappa light chain, secondary to a plasma cell dyscrasia
(2) aminoaciduria
(3) renal glycosuria
(4) phosphaturia
(5) renal tubular acidosis (RTA)
Diagnosis depends on:
(1) demonstrating Bence-Jones proteinuria
(2) presence of light chain crystals in the cytoplasm of proximal renal tubular epithelial cells, which are phosphotungstic acid-hematoxylin (PTAH) positive
(3) variable presence of light chain casts within the renal tubules
(4) exclusion of other causes of renal Fanconi syndrome
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