Screening and Differential Diagnosis of Renal Light Chain-Associated Diseases

Abstract

Background: Renal involvement in the light chain-associated diseases multiple myeloma (MM), amyloidosis (AL) and monoclonal immune position disease (MIDD) is common and differential diagnosis usually requires renal biopsy. The aim of this study was to investigate if noninvasive methods are viable to identify and differentiate between the various types of kidney diseases. Patients and Methods: All patients with a light chain-associated disease admitted to our center from 1996 to 2008 were retrospectively evaluated. Renal biopsy data were correlated with proteinuria findings. Results: Only the ratio of free ĸ/λ light chains showed a good sensitivity for myeloma cast nephropathy (MCN), AL and MIDD. The λ light chain was characteristic for AL, the ĸ light chain dominated in MIDD. Renal function at the time of diagnosis was worst in MIDD. MCN presented with a proteinuria of >3.5 g/g creatinine. In contrast, a higher proteinuria was found in AL or MIDD. Whereas the ĸ/λ ratio in the urine was pathological for all three diseases, extremely high or low ratios indicated the presence of MCN. However, in AL or MIDD, the ratio was only moderately elevated. Conclusion: A noninvasive differentiation between MCN and other forms of renal light chain disease is possible.

Multiple Myeloma Can Be Accurately Diagnosed in Acute Kidney Injury Patients Using a Rapid Serum Free Light Chain Test

“…Although absolute levels of κ and λ FLCs were significantly different between groups there was overlap in the distribution between myeloma and non-myeloma patient results. This is consistent with previous findings where only the κ:λ FLC ratio showed very good sensitivity for myeloma cast nephropathy [19]. Importantly, the portable device offered the same level of diagnostic performance previously reported for conventional laboratory-based testing.…”

The risk of renal disease is increased in lambda myeloma with bone marrow amyloid deposits

“…AL was found to be more common in lambda (80%) and monoclonal immunoglobulin deposition disease (MIDD) and even MM-cast nephropathy (MCN) in kappa MM among patients undergoing renal biopsy 4–6. Twenty-four-hour proteinuria was significantly higher in MM-associated amyloidosis compared with MIDD and MCN.…”

“…Concomitant AL in myeloma (AL-MM) is observed in 10–15% of patients 3. Renal disease in MM, when verified by kidney biopsy, is caused by amyloid deposits (AD) in 21–30% of patients 4–6. Renal function impairment is present in 50% (severe in 20%) of all MM patients at diagnosis 3…”

Renale Manifestationen von Leichtkettenerkrankungen - Epidemiologie und Prognose

“…Obwohl die Surrogatparameter, die als zusätzliche Einschlusskriterien für diese Gruppe fungierten, in einer vorangegangenen Untersuchung evaluiert wurden [15], kann letztlich nicht ausgeschlossen werden, dass auch Patienten in diese Gruppe gelangten, die gleichzeitig an einer AL-Amyloidose oder einer MIDD litten. Ähnlich kritisch ist die Zuordnung der Patienten zur AL-Amyloidose-Gruppe zu betrachten, die aufgrund einer extrarenalen Histologie in Kombination mit einer Proteinurie und einer eingeschränkten Nierenfunktion dieser Gruppe zugeordnet wurden.…”

“…Auch die Amyloidablagerung kann in Abhängigkeit des betroffenen Kompartiments eine glomeruläre bzw. tubuläre Proteinurie auslösen, die keine prominente Bence-Jones-Komponente aufweisen muss [15]. MIDD und AL-Amyloidose können extrarenale Ablagerungen monoklonaler Leichtketten verursachen, die im Falle der Herzbeteiligung prognoseentscheidend sein können [19,27,30].…”