PMID- 23461212
OWN - NLM
STAT- MEDLINE
DCOM- 20130903
LR  - 20131121
IS  - 0385-2385 (Print)
IS  - 0385-2385 (Linking)
VI  - 55
IP  - 1
DP  - 2013
TI  - [Autopsy case of light chain deposition disease presented by nephrotic syndrome].
PG  - 63-70
AB  - 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.
FAU - Suzuki, Soh
AU  - Suzuki S
AD  - Department of Internal Medicine, Tachikawa Sogo Hospital, Tokyo, Japan.
FAU - Yokota, Sho
AU  - Yokota S
FAU - Masuyama, Tomoyuki
AU  - Masuyama T
FAU - Ogihara, Hideki
AU  - Ogihara H
FAU - Ohishi, Manabu
AU  - Ohishi M
FAU - Katayama, Norimasa
AU  - Katayama N
FAU - Koizumi, Hiroshi
AU  - Koizumi H
FAU - Namiki, Masao
AU  - Namiki M
LA  - jpn
PT  - Case Reports
PT  - English Abstract
PT  - Journal Article
PT  - Review
PL  - Japan
TA  - Nihon Jinzo Gakkai Shi
JT  - Nihon Jinzo Gakkai shi
JID - 7505731
RN  - 0 (Immunoglobulin Light Chains)
RN  - 9PHQ9Y1OLM (Prednisolone)
RN  - Q41OR9510P (Melphalan)
SB  - IM
MH  - Aged, 80 and over
MH  - Autopsy
MH  - Humans
MH  - Immunoglobulin Light Chains/metabolism
MH  - Kidney/*ultrastructure
MH  - Male
MH  - Melphalan/therapeutic use
MH  - Microscopy, Electron
MH  - Multiple Myeloma/drug therapy/*pathology
MH  - Nephrotic Syndrome/diagnosis/*pathology
MH  - Prednisolone/therapeutic use
EDAT- 2013/03/07 06:00
MHDA- 2013/09/04 06:00
CRDT- 2013/03/07 06:00
PHST- 2013/03/07 06:00 [entrez]
PHST- 2013/03/07 06:00 [pubmed]
PHST- 2013/09/04 06:00 [medline]
PST - ppublish
SO  - Nihon Jinzo Gakkai Shi. 2013;55(1):63-70.