PMID- 28001187
OWN - NLM
STAT- MEDLINE
DCOM- 20180129
LR  - 20180129
IS  - 2175-8239 (Electronic)
IS  - 0101-2800 (Linking)
VI  - 38
IP  - 4
DP  - 2016 Dec
TI  - Treatment of idiopathic light chain deposition disease: complete remission with 
      bortezomib and dexamethasone.
PG  - 450-454
LID - S0101-28002016000400450 [pii]
LID - 10.5935/0101-2800.20160071 [doi]
AB  - Light chain deposition disease (LCDD) is a rare clinical entity characterized by 
      the deposition of light chain immunoglobulins in different tissues and primarily 
      affects the kidneys, followed by the liver and heart. This disease often 
      manifests as nephrotic syndrome with marked proteinuria and rapid deterioration 
      of renal function. More than 50% of cases are secondary to multiple myeloma or 
      other lymphoproliferative diseases, with a well-established treatment aimed at 
      controlling the underlying disease. In rare cases, there is no detection of an 
      associated hematological disease, referred to as idiopathic LCDD. In these cases, 
      there is no evidence-based consensus on the therapeutic approach, and management 
      is based on the clinical experience of reported cases. Here we report a case of 
      idiopathic LCDD treated with bortezomib and dexamethasone with complete 
      hematologic responses, significant reduction of proteinuria, and improved renal 
      function.
FAU - Souto Filho, Joao Tadeu Damian
AU  - Souto Filho JT
AD  - Faculdade de Medicina de Campos, Brazil.
FAU - Monteiro, Jorge Murilo Grillo
AU  - Monteiro JM
AD  - Faculdade de Medicina de Campos, Brazil.
FAU - Andrade, Inez Barcellos de
AU  - Andrade IB
AD  - Faculdade de Medicina de Campos, Brazil.
LA  - por
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - Brazil
TA  - J Bras Nefrol
JT  - Jornal brasileiro de nefrologia
JID - 9426946
RN  - 0 (Antineoplastic Agents)
RN  - 0 (Glucocorticoids)
RN  - 0 (Immunoglobulin Light Chains)
RN  - 69G8BD63PP (Bortezomib)
RN  - 7S5I7G3JQL (Dexamethasone)
SB  - IM
MH  - Antineoplastic Agents/*therapeutic use
MH  - Bortezomib/*therapeutic use
MH  - Dexamethasone/*therapeutic use
MH  - Glucocorticoids/*therapeutic use
MH  - Humans
MH  - *Immunoglobulin Light Chains
MH  - Male
MH  - Middle Aged
MH  - Paraproteinemias/*drug therapy
MH  - Remission Induction
EDAT- 2016/12/22 06:00
MHDA- 2018/01/30 06:00
CRDT- 2016/12/22 06:00
PHST- 2016/02/17 00:00 [received]
PHST- 2016/04/25 00:00 [accepted]
PHST- 2016/12/22 06:00 [entrez]
PHST- 2016/12/22 06:00 [pubmed]
PHST- 2018/01/30 06:00 [medline]
AID - S0101-28002016000400450 [pii]
AID - 10.5935/0101-2800.20160071 [doi]
PST - ppublish
SO  - J Bras Nefrol. 2016 Dec;38(4):450-454. doi: 10.5935/0101-2800.20160071.