PMID- 29393843
OWN - NLM
STAT- MEDLINE
DCOM- 20180921
LR  - 20190318
IS  - 0301-0430 (Print)
IS  - 0301-0430 (Linking)
VI  - 89
IP  - 6
DP  - 2018 Jun
TI  - Successful treatment of nephrotic syndrome induced by lambda light chain 
      deposition disease using lenalidomide: A case report and review of the 
      literature
.
PG  - 461-468
LID - 10.5414/CN109342 [doi]
AB  - BACKGROUND: Light chain deposition disease (LCDD) is a monoclonal immunoglobulin 
      deposition disease (MIDD) that is characterized by the deposition of monoclonal 
      light chains in multiple organs, including the kidney. It is a rare disorder 
      caused by an underlying monoclonal plasma cell dyscrasia. LCDD with renal 
      involvement causes proteinuria, which sometimes can lead to nephrotic syndrome. 
      The monoclonal light chains are mostly in the kappa form. Treatment of LCDD is the 
      same as that for multiple myeloma (MM); however, some conventional anticancer 
      drugs show substantial toxicity and therefore cannot be administered to older 
      patients or those with renal impairment. CASE PRESENTATION: An 80-year-old woman 
      was referred to our department with severe nephrotic syndrome (13.6 g/gCr) and 
      anemia. A renal biopsy showed mesangial proliferation and mesangial matrix 
      expansion, and immunohistochemistry showed positive staining for lambda chains along 
      the glomerular basement membrane, but was negative for kappa chains or amyloid 
      deposition. A bone marrow biopsy revealed 64% plasma cells. Immunoglobulin G 
      (IgG)-lambda type M protein was detected, and the levels of free lambda chain was 
      significantly increased. We concluded that her nephrotic syndrome was caused by 
      LCDD, which resulted from IgG-lambda MM. The induction of a BCD<bold> 
      </bold>(bortezomib, cyclophosphamide, and dexamethasone) treatment regimen did 
      not lead to a hematological response or decrease in proteinuria. The 
      administration of combination therapy of lenalidomide and prednisolone led to the 
      successful reduction of proteinuria and hematuria. CONCLUSIONS: We presented a 
      very rare case report describing the successful treatment of LCDD (lambda 
      chain)-induced nephrotic syndrome with lenalidomide.
.
FAU - Mima, Akira
AU  - Mima A
FAU - Nagahara, Dai
AU  - Nagahara D
FAU - Tansho, Kosuke
AU  - Tansho K
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - Germany
TA  - Clin Nephrol
JT  - Clinical nephrology
JID - 0364441
RN  - 0 (Immunoglobulin lambda-Chains)
RN  - 0 (Immunologic Factors)
RN  - 4Z8R6ORS6L (Thalidomide)
RN  - F0P408N6V4 (Lenalidomide)
SB  - IM
MH  - Aged, 80 and over
MH  - Female
MH  - Humans
MH  - Immunoglobulin lambda-Chains/*metabolism
MH  - Immunologic Factors/*therapeutic use
MH  - Lenalidomide
MH  - Multiple Myeloma/*complications
MH  - *Nephrotic Syndrome/drug therapy/etiology
MH  - Thalidomide/*analogs & derivatives/therapeutic use
EDAT- 2018/02/03 06:00
MHDA- 2018/09/22 06:00
CRDT- 2018/02/03 06:00
PHST- 2018/05/23 00:00 [accepted]
PHST- 2018/02/03 06:00 [pubmed]
PHST- 2018/09/22 06:00 [medline]
PHST- 2018/02/03 06:00 [entrez]
AID - 16594 [pii]
AID - 10.5414/CN109342 [doi]
PST - ppublish
SO  - Clin Nephrol. 2018 Jun;89(6):461-468. doi: 10.5414/CN109342.