LIGHT CHAIN CAST NEPHROPATHY IN A PATIENT WITH MULTIPLE MYELOMA AND LOW SERUM FREE LIGHT CHAIN CONCETRATION

 

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https://storage.unitedwebnetwork.com/files/1099/395bee57091987689f2095b92c8e559d.pdf
LIGHT CHAIN CAST NEPHROPATHY IN A PATIENT WITH MULTIPLE MYELOMA AND LOW SERUM FREE LIGHT CHAIN CONCETRATION

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Eleni
Theodoropoulou
Konstantinos Rizogiannis k.rizogiannis@hotmail.com Alexandra General Hospital Nephrology Athens Greece -
Harikleia Gakiopoulou charagak28@gmail.com 1st Pathology Department School of Medicine, National and Kapodistrian University of Athens Athens Greece -
Paraskevi Tseke tsekedaki@gmail.com Alexandra General Hospital Nephrology Athens Greece -
Erasmia Psimenou erasmia7@otenet.gr Alexandra General Hospital Nephrology Athens Greece -
Eleni Theodoropoulou htheodoropoulou@yahoo.gr Alexandra General Hospital Nephrology Athens Greece *
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Light chain cast nephropathy is a kidney disorder primarily associated with myeloma. A heavy burden of filtered free light chains/Bence Jones protein, cause obstructive tubulopathy and/or direct toxicity promoting acute kidney injury and rapidly progressive renal failure. Factors, which may precipitate this phenomenon are dehydration, hypercalcemia, high-dose diuretics and nephrotoxic drugs (contrast agents, NSAIDs).

A 60-year-old female patient with a history of multiple myeloma (IgGκ) in partial remission under treatment presented markedly dehydrated with weakness, debilitation, low blood pressure, and reduced urine output. The last 7 days she had been suffering gastroenteritis and diarrhea.

At presentation, serum creatinine (Cr) was 16.3 mg/dl, Hct 21,7%, calcium 7.7mg/dl. Stool sample examination (PCR) and culture for enteric pathogens was negative. Serum protein electrophoresis (SPEP), immunofixation electrophoresis (IFE) and quantitative determination of serum immunoglobulins were normal (κ: 0.73mg/L, λ:0.09 mg/L) albeit κ-light chains were increased in urine (κ : 203 mg/L).

A renal biopsy was performed which showed the presence of eosinophilic, fragmented casts in the tubular lumen, exhibiting irregular, angulated, and geometric shapes with accumulation of inflammatory cells in their marginal parts. On immunofluorescence, intense positivity for κ-light chains in the intratubular casts was observed along with moderate fixation of monoclonal IgG while λ-light chains were negative. Focal interstitial fibrosis and mild infiltration by inflammatory cells (lymphocytes and macrophages) were also depicted.

Light-chain cast nephropathy was diagnosed.

The patient received parenteral fluid support while she was in need for a few haemodialysis treatments. The urine output gradually increased and kidney function improved. 

Light chain cast nephropathy is a common cause of severe renal impairment in patients with multiple myeloma. The serum free light chain assay is used in patients with unexplained acute kidney injury to screen for potential myeloma related cast nephropathy. However, the range of free light chains at which cast nephropathy can occur is uncertain. The International Myeloma Working Group associated renal impairment as a consequence of myeloma cast nephropathy to a serum free light chain level above 1500mg/L, whereas the International Kidney and Monoclonal Gammopathy Research Group proposed a serum free light chain level above 500mg/L. Surprisingly, our patient had low serum free light chain (κ: 0.73mg/L), which made the diagnosis of light chain cast nephropathy improbable. Diarrheal syndrome and dehydration seemed to have played a pivotal role in the precipitation of casts in this case.

Kewords