A case of the Light Chain Disease Associated with the Combined Light Chain Nephropathy

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A 34 year old male was admitted to the hospital with polydipsia, polyuria, severe bone pain and deformity of thorax. Twelve years previously, glycosuria and proteinuria had been established.He had a severe Bence-Jones proteinuria. The presence of κ-type light chain in serum and urine was demonstrated by tne immunoelectrophoreses. Residual immunoglobulins were markidly decreased. However, the percentage of plasma cells in the bone marrow was less than 10% and few atypical plasma cells were found. Therefore, it seems likely that he has not yet developed multiple myeloma but has been in the premyelomatic state.He had also the Fanconi's syndrome, the distal renal tubular acidosis and the renal diabetes inspidus.Therefore, the diagnosis of the light chain (κ) disease associated with the combined light chain nephropathy was made. Until recently only 8 cases of the combined light chain nephropathy (Smithline, 1976) have been reported in literatures.
日本臨床免疫学会の論文