title: Reversible left ventricular dysfunction and acute kidney injury in a patient with nonamyloid light chain deposition disease.
abstract: Non-amyloid light chain deposition disease (LCDD) is a rare entity that most commonly presents as proteinuria and/or renal dysfunction. We report on a patient who initially presented with acutely decompensated heart failure and subsequently developed nephrotic range proteinuria with attendant advanced renal dysfunction. The diagnosis of LCCD was made on renal biopsy.She was treated with five cycles of bortezomib and dexamethasone followed by cyclophosphamide priming for peripheral blood stem cell (PBSC) mobilization and auto logousstem cell transplant (ASCT). Four years later, she remains in very good partial response (VGPR) with a left ventricular ejection fraction (LVEF) of 58% and serum creatinine of 1.1 mg/dl. This observation supports the approach of aggressive management of patients with LCDD who have multiple organ failure.
authors.affiliation: Divisions of Nephrology, Cardiology, Oncology, Department of Medicine and Department of Pathology, School of Medicine, State University of New York at Stony Brook, NY 11794, USA. nand.wadhwa@sbumed.org
authors.forename: Nand K
authors.initials: NK
authors.lastname: Wadhwa
authors.forename: Amit
authors.initials: A
authors.lastname: Kamra
authors.forename: Hal A
authors.initials: HA
authors.lastname: Skopicki
authors.forename: Shambavi
authors.initials: S
authors.lastname: Richard
authors.forename: Frederick
authors.initials: F
authors.lastname: Miller
authors.forename: Edward P
authors.initials: EP
authors.lastname: Nord
mesh_terms: D058186:Acute Kidney Injury / Q000209:etiology*; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000378:metabolism*; D008875:Middle Aged; D010265:Paraproteinemias / Q000150:complications*; D014182:Transplantation, Autologous; D018487:Ventricular Dysfunction, Left / Q000209:etiology*
chemical_list: D007147:Immunoglobulin Light Chains