PMID- 22573235
OWN - NLM
STAT- MEDLINE
DCOM- 20121107
LR  - 20211021
IS  - 1865-3774 (Electronic)
IS  - 0925-5710 (Linking)
VI  - 96
IP  - 1
DP  - 2012 Jul
TI  - Light chain deposition disease affecting the gastrointestinal tract in the 
      setting of post-living donor kidney transplantation.
PG  - 125-31
LID - 10.1007/s12185-012-1090-1 [doi]
AB  - Light chain deposition disease (LCDD) is an uncommon, clonal plasma cell 
      proliferative disorder, in which monoclonal immunoglobulin light chains deposit 
      in various tissues, resulting in organ dysfunction. Gastrointestinal (GI) 
      involvement has been described in both primary and secondary amyloidosis, but has 
      rarely been reported in LCDD, and only as an incidental finding. We report a case 
      of LCDD in living related kidney transplant recipient presenting with severe GI 
      dysmotility, weight loss and progressive allograft dysfunction. A diagnosis of 
      LCDD was based on the kidney biopsy findings in the failing renal allograft, 
      along with the presence of excess serum free kappa light chains and abnormal 
      kappa:lambda ratio. Subsequent review of GI biopsies confirmed kappa light chain 
      immunoglobulin deposition within the stomach. Further investigation suggested 
      additional hepatic and cardiac involvement. The patient went on to receive 
      bortezomib, achieving a biochemical response and stabilization of his advanced 
      renal dysfunction; however, bortezomib was discontinued due to toxicity. The 
      patient was subsequently treated with lenalidomide and dexamethasone, which were 
      better tolerated. Further biochemical response and resolution of the GI symptoms 
      was observed after 10 months of treatment. In summary, we present the first case 
      of LCDD with symptomatic GI involvement, in which the diagnosis was established 
      by intestinal biopsies. Our report also highlights the feasibility and 
      effectiveness of lenalidomide in the treatment of LCDD.
FAU - Jimenez-Zepeda, Victor H
AU  - Jimenez-Zepeda VH
AD  - Department of Medical Oncology and Hematology, Princess Margaret Hospital, 620 
      University Avenue, Toronto, Ontario, Canada. victor.zepeda@uhn.on.ca
FAU - Vajpeyi, Rajkumar
AU  - Vajpeyi R
FAU - John, Rohan
AU  - John R
FAU - Trudel, Suzanne
AU  - Trudel S
LA  - eng
PT  - Case Reports
PT  - Journal Article
DEP - 20120510
PL  - Japan
TA  - Int J Hematol
JT  - International journal of hematology
JID - 9111627
RN  - 0 (Antineoplastic Agents)
RN  - 0 (Boronic Acids)
RN  - 0 (Immunoglobulin Light Chains)
RN  - 0 (Immunoglobulin kappa-Chains)
RN  - 0 (Immunoglobulin lambda-Chains)
RN  - 0 (Pyrazines)
RN  - 4Z8R6ORS6L (Thalidomide)
RN  - 69G8BD63PP (Bortezomib)
RN  - 7S5I7G3JQL (Dexamethasone)
RN  - F0P408N6V4 (Lenalidomide)
SB  - IM
MH  - Antineoplastic Agents/therapeutic use
MH  - Boronic Acids/administration & dosage/therapeutic use
MH  - Bortezomib
MH  - Dexamethasone/administration & dosage/therapeutic use
MH  - Gastrointestinal Tract/*immunology/pathology
MH  - Humans
MH  - Immunoglobulin Light Chains/blood/*immunology
MH  - Immunoglobulin kappa-Chains/blood/immunology
MH  - Immunoglobulin lambda-Chains/blood/immunology
MH  - Kidney Transplantation/*pathology
MH  - Lenalidomide
MH  - Male
MH  - Middle Aged
MH  - Paraproteinemias/*diagnosis/drug therapy/*immunology
MH  - Pyrazines/administration & dosage/therapeutic use
MH  - Thalidomide/administration & dosage/analogs & derivatives/therapeutic use
MH  - Treatment Outcome
EDAT- 2012/05/11 06:00
MHDA- 2012/11/08 06:00
CRDT- 2012/05/11 06:00
PHST- 2011/11/25 00:00 [received]
PHST- 2012/04/19 00:00 [accepted]
PHST- 2012/04/18 00:00 [revised]
PHST- 2012/05/11 06:00 [entrez]
PHST- 2012/05/11 06:00 [pubmed]
PHST- 2012/11/08 06:00 [medline]
AID - 10.1007/s12185-012-1090-1 [doi]
PST - ppublish
SO  - Int J Hematol. 2012 Jul;96(1):125-31. doi: 10.1007/s12185-012-1090-1. Epub 2012 
      May 10.