Paraprotein–Related Kidney Disease: Evaluation and Treatment of Myeloma Cast Nephropathy

Abstract

Nearly 50% of patients with multiple myeloma develop renal disease, most commonly from AKI caused by cast nephropathy. Development of AKI is associated with poor 1-year survival and reduces the therapeutic options available to patients. There is a great need for more effective therapies. Cast nephropathy is caused by the interaction and aggregation of filtered free light chains and Tamm-Horsfall protein causing intratubular obstruction and damage. The key to treating cast nephropathy is rapid lowering of free light chains, because this correlates with renal recovery. Newer chemotherapy agents rapidly lower free light chains and have been referred to as renoprotective. There is additional great interest in using extracorporeal therapies to remove serum free light chains. Small trials initially showed benefit of therapeutic plasma exchange to improve renal outcomes in cast nephropathy, but a large randomized trial of therapeutic plasma exchange failed to show benefit. A newer technique is extended high-cutoff hemodialysis. This modality uses a high molecular weight cutoff filter to remove free light chains. To date, trials of high-cutoff hemodialysis use in patients with cast nephropathy have been encouraging. However, there are no randomized trials showing the benefit of high-cutoff hemodialysis when used in addition to newer chemotherapeutic regimens. Until these studies are available, high-cutoff hemodialysis cannot be recommended as standard of care.

Clinicopathologic predictors of renal outcomes in light chain cast nephropathy: a multicenter retrospective study

“…However, because improvement in renal function usually occurs within the first 6 months, a lead-time bias is unlikely. 29,35,49 Our multivariate model indicates that the best eGFR during follow-up is more relevant than the presenting one. Figure 5A shows that achievement of renal response according to IMWG criteria was associated with higher OS.…”

Multiple Myeloma Mimicking a Small Vessel Vasculitis Presentation

“…With the diagnosis of cast nephropathy secondary to multiple myeloma, chemotherapy was initiated. ere is also a possible clinical benefit removing free light chains by extracorporeal treatment [5][6][7][8][9][10][11]. For this reason, plasmapheresis was maintained until the initially planned 7 sessions were completed.…”

Nefropatías secundarias a paraproteína: perfil clínico a partir de hallazgos histológicos

“…El compromiso histopatológico renal más frecuente fue amiloidosis renal, y luego, el RM. Esto se debe a una subrepresentación del RM, ya que cuando el diagnóstico de MM y proteinuria por cadenas livianas son evidentes, es posible prescindir de la biopsia renal 23 . Sin embargo, la nefropatía asociada a paraproteínas sensiblemente más frecuente es el RM 5 .…”