title: [Autopsy case of light chain deposition disease presented by nephrotic syndrome].
abstract: An eighty-one-year-old male with lower leg edema and proteinuria was diagnosed as having nephrotic syndrome and was hospitalized for a detailed examination. Kidney biopsy and immunostaining revealed nodular glomerulosclerosis and deposition of lambda chains, respectively. Because these findings indicate the occurrence of light chain deposition disease (LCDD), the underlying disease was found to be multiple myeloma BJ-lambda. After the administration of melphalan and prednisolone, followed by further addition of zoledronic acid, the patient's nephrosis remitted. However, renal dysfunction gradually deteriorated further and hemodialysis was instituted. He eventually died of gastrointestinal bleeding and biliary infection. The period of time from the initial diagnosis to death was thirty months. Autopsy revealed pervasive infiltration of plasma cells and light chain deposition in multiple organs. The uncontrollable gastrointestinal bleeding appears to have been caused by light chain deposition in the vascular walls of a bile duct. Although medical treatment for elderly LCDD cases depends on chemotherapy alone, it is difficult to obtain a complete remission with melphalan and prednisolone, according to the literature. Reports on the validities of biological agents, such as bortezomib, are beginning to appear, and accumulation of further therapeutic experience is eagerly awaited.
authors: Department of Internal Medicine, Tachikawa Sogo Hospital, Tokyo, Japan.
authors: Soh
authors: S
authors: Suzuki
authors: Sho
authors: S
authors: Yokota
authors: Tomoyuki
authors: T
authors: Masuyama
authors: Hideki
authors: H
authors: Ogihara
authors: Manabu
authors: M
authors: Ohishi
authors: Norimasa
authors: N
authors: Katayama
authors: Hiroshi
authors: H
authors: Koizumi
authors: Masao
authors: M
authors: Namiki
mesh_terms: D000369:Aged, 80 and over; D001344:Autopsy; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000378:metabolism; D007668:Kidney / Q000648:ultrastructure*; D008297:Male; D008558:Melphalan / Q000627:therapeutic use; D008854:Microscopy, Electron; D009101:Multiple Myeloma / Q000188:drug therapy / Q000473:pathology*; D009404:Nephrotic Syndrome / Q000175:diagnosis / Q000473:pathology*; D011239:Prednisolone / Q000627:therapeutic use
chemical_list: D007147:Immunoglobulin Light Chains; D011239:Prednisolone; D008558:Melphalan