PMID- 26392598
OWN - NLM
STAT- MEDLINE
DCOM- 20160503
LR  - 20220331
IS  - 1528-0020 (Electronic)
IS  - 0006-4971 (Print)
IS  - 0006-4971 (Linking)
VI  - 126
IP  - 26
DP  - 2015 Dec 24
TI  - Natural history and outcome of light chain deposition disease.
PG  - 2805-10
LID - 10.1182/blood-2015-07-658872 [doi]
AB  - Light chain deposition disease (LCDD) is characterized by the deposition of 
      monotypic immunoglobulin light chains in the kidney, resulting in renal 
      dysfunction. Fifty-three patients with biopsy-proven LCDD were prospectively 
      followed at the UK National Amyloidosis Center. Median age at diagnosis was 56 
      years, and patients were followed for a median of 6.2 years (range, 1.1-14.0 
      years). Median renal survival from diagnosis by Kaplan-Meier analysis was 5.4 
      years, and median estimated patient survival was 14.0 years; 64% of patients were 
      alive at censor. Sixty-two percent of patients required dialysis, and median 
      survival from commencement of dialysis was 5.2 years. There was a strong 
      association between hematologic response to chemotherapy and renal outcome, with 
      a mean improvement in glomerular filtration rate (GFR) of 6.1 mL/min/year among 
      those achieving a complete or very good partial hematologic response (VGPR) with 
      chemotherapy, most of whom remained dialysis independent, compared with a mean 
      GFR loss of 6.5 mL/min/year among those achieving only a partial or no 
      hematologic response (P < .009), most of whom developed end-stage renal disease 
      (ESRD; P = .005). Seven patients received a renal transplant, and among those 
      whose underlying clonal disorder was in sustained remission, there was no 
      recurrence of LCDD up to 9.7 years later. This study highlights the need to 
      diagnose and treat LCDD early and to target at least a hematologic VGPR with 
      chemotherapy, even among patients with advanced renal dysfunction, to delay 
      progression to ESRD and prevent recurrence of LCDD in the renal allografts of 
      those who subsequently receive a kidney transplant.
CI  - (c) 2015 by The American Society of Hematology.
FAU - Sayed, Rabya H
AU  - Sayed RH
AD  - National Amyloidosis Centre and Centre for Nephrology, Division of Medicine, 
      University College London, London, United Kingdom.
FAU - Wechalekar, Ashutosh D
AU  - Wechalekar AD
AD  - National Amyloidosis Centre and.
FAU - Gilbertson, Janet A
AU  - Gilbertson JA
AD  - National Amyloidosis Centre and.
FAU - Bass, Paul
AU  - Bass P
AD  - Centre for Nephrology, Division of Medicine, University College London, London, 
      United Kingdom.
FAU - Mahmood, Shameem
AU  - Mahmood S
AD  - National Amyloidosis Centre and.
FAU - Sachchithanantham, Sajitha
AU  - Sachchithanantham S
AD  - National Amyloidosis Centre and.
FAU - Fontana, Marianna
AU  - Fontana M
AD  - National Amyloidosis Centre and.
FAU - Patel, Ketna
AU  - Patel K
AD  - National Amyloidosis Centre and.
FAU - Whelan, Carol J
AU  - Whelan CJ
AD  - National Amyloidosis Centre and.
FAU - Lachmann, Helen J
AU  - Lachmann HJ
AD  - National Amyloidosis Centre and.
FAU - Hawkins, Philip N
AU  - Hawkins PN
AD  - National Amyloidosis Centre and.
FAU - Gillmore, Julian D
AU  - Gillmore JD
AD  - National Amyloidosis Centre and.
LA  - eng
PT  - Journal Article
DEP - 20150921
PL  - United States
TA  - Blood
JT  - Blood
JID - 7603509
RN  - 0 (Immunoglobulin Light Chains)
SB  - IM
CIN - Blood. 2015 Dec 24;126(26):2770-1. PMID: 26705338
MH  - Female
MH  - Humans
MH  - *Immunoglobulin Light Chains
MH  - Kaplan-Meier Estimate
MH  - Kidney Failure, Chronic/*etiology/mortality/pathology
MH  - Male
MH  - Middle Aged
MH  - Paraproteinemias/mortality/*pathology/therapy
PMC - PMC4732758
EDAT- 2015/09/24 06:00
MHDA- 2016/05/04 06:00
PMCR- 2015/12/24
CRDT- 2015/09/23 06:00
PHST- 2015/07/17 00:00 [received]
PHST- 2015/09/16 00:00 [accepted]
PHST- 2015/09/23 06:00 [entrez]
PHST- 2015/09/24 06:00 [pubmed]
PHST- 2016/05/04 06:00 [medline]
PHST- 2015/12/24 00:00 [pmc-release]
AID - S0006-4971(20)34812-6 [pii]
AID - 2015/658872 [pii]
AID - 10.1182/blood-2015-07-658872 [doi]
PST - ppublish
SO  - Blood. 2015 Dec 24;126(26):2805-10. doi: 10.1182/blood-2015-07-658872. Epub 2015 
      Sep 21.