Bortezomib-based chemotherapy for light chain deposition disease presenting as acute renal failure

Abstract

We report a case of kappa light chain deposition disease (LCDD) associated with multiple myeloma in a patient presenting with acute renal failure, 2+ proteinuria and hypercalcemia. Serum protein electrophoresis showed an M-spike at 0.1 g/dL. 24-h urine protein electrophoresis showed Bence-Jones proteinuria of 3.8 g. Serum-free light chain assay found excess kappa chains at 3080 mg/L, with normal lambda and an elevated kappa:lambda ratio of 124.7. A kidney biopsy revealed kappa light chain nephropathy with PAS-negative tubular casts in the cortex and outer medulla. Bone marrow biopsy showed 15% kappa-restricted plasma cells. Serum beta-2 microglobulin level was elevated at 7.94 mg/dL. The patient received a 3-day course of plasmapheresis followed by eight cycles of bortezomib (Velcade®), liposomal doxorubicin, and dexamethasone (VDD) and did not require hemodialysis. As partial response was not achieved, treatment was continued with three cycles of bortezomib, cyclophosphamide, dexamethasone, and thalidomide, followed by thalidomide maintenance at 100 mg daily. Thirty-two months after the diagnosis, the patient's renal function was improved and he achieved a partial response. This case underlines the feasibility and effectiveness of bortezomib-based chemotherapy in the treatment of LCDD with severe renal dysfunction.

Liver Failure Caused by Light Chain Deposition Disease Associated with Multiple Myeloma

“…Excretion of hydrogen ion and potassium will be impaired if the light chain deposits involves the tubules (17). Kidney function prognosis in MM patients with LCDD is dismal despite reports showing a reduction of proteinuria and stabilization of renal function in several uncontrolled studies and case reports (18)(19)(20). Most such cases end up rapidly developing end-stage renal disease (ESRD).…”

Light-Chain Deposition Disease Successfully Treated with Bortezomib in an Elderly Patient: A Case Report and Review of the Literature

“…Bortezomib has been suggested to inhibit NF-κB, thereby decreasing TGF-β levels and collagen production, eventually preventing glomerulosclerosis and renal dysfunction (11)(12)(13). Furthermore, several case reports have already shown that bortezomib stabilizes the renal function in patients with LCDD without serious adverse effects (19)(20)(21)(22)(23)(24). Based on these data and those obtained from reports, it was decided to administer BD therapy to this elderly patient.…”

Light Chain Deposition Disease in an Older Adult Patient Successfully Treated with Long-term Administration of Bortezomib, Melphalan and Prednisone

“…Similar results have been reported among LCDD patients. Table shows a list of previous reports describing patients with LCDD who received bortezomib-based regimens without high-dose chemotherapy (11,14,15). The patient described here showed an abrupt elevation in the serum levels of hepatobiliary enzymes after the first cycle of the VCD regimen.…”