PMID- 30755931
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20220331
IS  - 2284-2594 (Electronic)
IS  - 2284-2594 (Linking)
VI  - 4
IP  - 3
DP  - 2017
TI  - Light-Chain Deposition Disease with Prominent Hepatic Involvement.
PG  - 000545
LID - 10.12890/2017_000545 [doi]
LID - 000545
AB  - Light-chain deposition disease (LCDD) is a rare monoclonal gammopathy that 
      involves the deposition of light chains (LC) in multiple organs, leading to 
      progressive dysfunction. The kidney is usually the most affected organ and 
      responsible for the initial clinical manifestations. We present the case of a 
      patient with LCDD with prominent liver involvement (marked cholestasis, 
      hepatomegaly and portal hypertension) but with no evidence of coexisting 
      lymphoproliferative disorder. LEARNING POINTS: Light-chain deposition disease 
      (LCDD) is rare, and diagnosis can be challenging, especially in the absence of a 
      coexistent lymphoproliferative disorder. The presence of light chains in blood or 
      urine can be intermittent, and immunohistochemistry results of biopsy can be 
      difficult to interpret. Hence, there is a need for a high level of suspicion in 
      this diagnosis.Although renal involvement is the most classic presentation, LCDD 
      should be suspected when there is multiple organ involvement, as in this case, 
      even when renal manifestations do not dominate the clinical picture.Prognosis 
      depends on the affected organs, and treatment should be started as rapidly as 
      possible to suppress production of the clonal light-chain and halt organ damage.
FAU - Cristino, Ana
AU  - Cristino A
AD  - Servico de Medicina Interna, Centro Hospitalar de Tras-os-Montes e Alto Douro, 
      Vila Real, Portugal.
FAU - Pais, Carmen
AU  - Pais C
AD  - Servico de Medicina Interna, Centro Hospitalar de Tras-os-Montes e Alto Douro, 
      Vila Real, Portugal.
FAU - Silva, Renata
AU  - Silva R
AD  - Servico de Medicina Interna, Centro Hospitalar de Tras-os-Montes e Alto Douro, 
      Vila Real, Portugal.
FAU - Carrola, Paulo
AU  - Carrola P
AD  - Servico de Medicina Interna, Centro Hospitalar de Tras-os-Montes e Alto Douro, 
      Vila Real, Portugal.
LA  - eng
PT  - Journal Article
DEP - 20170427
PL  - Italy
TA  - Eur J Case Rep Intern Med
JT  - European journal of case reports in internal medicine
JID - 101648453
PMC - PMC6346872
OTO - NOTNLM
OT  - Light-chain deposition
OT  - monoclonal
OT  - plasma cell
COIS- Conflicts of Interests: The Authors declare that there are no competing 
      interests.
EDAT- 2017/04/27 00:00
MHDA- 2017/04/27 00:01
PMCR- 2017/04/27
CRDT- 2019/02/14 06:00
PHST- 2016/12/01 00:00 [received]
PHST- 2016/12/16 00:00 [accepted]
PHST- 2019/02/14 06:00 [entrez]
PHST- 2017/04/27 00:00 [pubmed]
PHST- 2017/04/27 00:01 [medline]
PHST- 2017/04/27 00:00 [pmc-release]
AID - 545-1-3430-1-10-20170118 [pii]
AID - 10.12890/2017_000545 [doi]
PST - epublish
SO  - Eur J Case Rep Intern Med. 2017 Apr 27;4(3):000545. doi: 10.12890/2017_000545. 
      eCollection 2017.