PMID- 17578193
OWN - NLM
STAT- MEDLINE
DCOM- 20070814
LR  - 20110801
IS  - 0385-2385 (Print)
IS  - 0385-2385 (Linking)
VI  - 49
IP  - 4
DP  - 2007
TI  - [A case of acute renal and liver dysfunction with light chain deposition 
      disease].
PG  - 459-63
AB  - A 62 year old woman was referred to our hospital because of acute renal and liver 
      dysfunction. Prior to admission, she had already been started on hemodyalysis 
      filtration(HDF). She showed facial edema and lumbar pain caused by an Ll 
      compressive fracture. Laboratory examinations revealed hypercalcemia (13.2 
      mg/dL), hyperammonemia (297 microg/dL) and her serum creatinine, blood urea 
      nitrogen and total bilirubin levels were 3.9 mg/dL, 37.4 mg/dL and 3.2 mg/dL, 
      respectively. Among the components of immunoglobulin, IgA was increased, while 
      IgG and IgM were decreased. Serum immunoelectrophoresis revealed the presence of 
      the IgA kappa type of M component. Punched out lesions were noted on her head 
      radiography. Severe plasmacytosis (60-70 % of total cells) were observed by a 
      bone marrow aspiration test, indicating the diagnosis of multiple myeloma. 
      Steroid pulse therapy was started with dexamethasone (40 mg/day, 3 days), and 
      plasma exchange was performed 8 times with continuous HDF. These treatments 
      failed to control hemodynamics and she died of disseminated intravascular 
      coagulation (DIC). Autopsy demonstrated amyloid-like depositions in 
      perisinusoidal space in the liver. In the kidney, there were nodular lesions in 
      the glomeruli, and depositions in the basement membrane of the uriniferous 
      tubuli. Congo red staining of these organs for amyloid yielded negative results. 
      Immunohistochemical staining gave positive results for IgA and kappa. Electron 
      microscopy revealed granular electron deposits in the glomeruli and tubular 
      basement membrane as well. Taken altogether, the diagnosis of the patient could 
      be light chain deposition disease (LCDD).
FAU - Ichikawa, Akiko
AU  - Ichikawa A
AD  - Department of Internal Medicine, Ohkubo Hospital-Tokyo Metropolitan Health and 
      Medical Treatment Corporation, Tokyo, Japan.
FAU - Hashimoto, Azusa
AU  - Hashimoto A
FAU - Shimizu, Ari
AU  - Shimizu A
FAU - Shizuku, Junnichi
AU  - Shizuku J
FAU - Abe, Yasutomo
AU  - Abe Y
FAU - Wakai, Sachiko
AU  - Wakai S
FAU - Ogura, Mitsuo
AU  - Ogura M
FAU - Ogawa, Tetsuya
AU  - Ogawa T
FAU - Nitta, Kosaku
AU  - Nitta K
LA  - jpn
PT  - Case Reports
PT  - English Abstract
PT  - Journal Article
PL  - Japan
TA  - Nihon Jinzo Gakkai Shi
JT  - Nihon Jinzo Gakkai shi
JID - 7505731
RN  - 0 (Immunoglobulin Light Chains)
RN  - 0 (Immunoglobulin kappa-Chains)
SB  - IM
MH  - Female
MH  - Humans
MH  - Immunoglobulin Light Chains/*metabolism
MH  - Immunoglobulin kappa-Chains/*metabolism
MH  - Kidney Diseases/*complications/metabolism/therapy
MH  - Liver Diseases/*complications
MH  - Middle Aged
MH  - Multiple Myeloma/diagnosis/metabolism
MH  - Renal Dialysis
EDAT- 2007/06/21 09:00
MHDA- 2007/08/19 09:00
CRDT- 2007/06/21 09:00
PHST- 2007/06/21 09:00 [pubmed]
PHST- 2007/08/19 09:00 [medline]
PHST- 2007/06/21 09:00 [entrez]
PST - ppublish
SO  - Nihon Jinzo Gakkai Shi. 2007;49(4):459-63.