title: 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.
authors.affiliation: Johns Hopkins University/Sinai Hospital Program of Internal Medicine, Baltimore, MD, USA.
authors.forename: Helen
authors.initials: H
authors.lastname: Gharwan
authors.forename: Cristina I
authors.initials: CI
authors.lastname: Truica
mesh_terms: D058186:Acute Kidney Injury / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy*; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D001897:Boronic Acids / Q000627:therapeutic use*; D000069286:Bortezomib; D003937:Diagnosis, Differential; D006801:Humans; D007145:Immunoglobulin kappa-Chains / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D009101:Multiple Myeloma / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy*; D011379:Prognosis; D011719:Pyrazines / Q000627:therapeutic use*; D012074:Remission Induction
chemical_list: D000970:Antineoplastic Agents; D001897:Boronic Acids; D007145:Immunoglobulin kappa-Chains; D011719:Pyrazines; D000069286:Bortezomib