title: Light chain deposition disease: novel biological insights and treatment advances.
abstract: Light chain deposition disease (LCDD) is a monoclonal gammopathy characterized by nonamyloid deposition of immunoglobulin light chains in various organs. Most cases present with renal dysfunction, a ubiquitous feature of this disease, and in some instances, it may progress to end-stage renal disease. Unfortunately, until now, no standard treatment has been established. The use of alkylating agents and steroids has been extensively reported. However, conventional chemotherapy response is generally limited with minor effects on kidney function. The use of novel agents such as bortezomib has shown a more rapid response with a dramatically important reduction of light chains in serum and/or urine in small series of cases. Furthermore, autologous stem cell transplantation has been reported as a feasible strategy in LCDD, able to prolong the dialysis-free survival. Nonetheless, toxicity from these therapies should be considered carefully because most of patients might present with kidney dysfunction that could limit the use of some agents.
authors.affiliation: Department of Medical Oncology and Hematology, Princess Margaret Hospital, Toronto, ON, Canada. victor.zepeda@uhn.ca
authors.forename: V H
authors.initials: VH
authors.lastname: Jimenez-Zepeda
mesh_terms: D001897:Boronic Acids / Q000627:therapeutic use; D000069286:Bortezomib; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000378:metabolism*; D007676:Kidney Failure, Chronic / Q000150:complications / Q000188:drug therapy / Q000503:physiopathology* / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D009102:Multiple Organ Failure / Q000150:complications / Q000188:drug therapy / Q000503:physiopathology* / Q000628:therapy*; D011719:Pyrazines / Q000627:therapeutic use
chemical_list: D001897:Boronic Acids; D007147:Immunoglobulin Light Chains; D011719:Pyrazines; D000069286:Bortezomib