PMID- 11434361
OWN - NLM
STAT- MEDLINE
DCOM- 20011101
LR  - 20220419
IS  - 0301-0430 (Print)
IS  - 0301-0430 (Linking)
VI  - 55
IP  - 6
DP  - 2001 Jun
TI  - Association of a POEMS syndrome and light chain deposit disease: first case 
      report.
PG  - 482-6
AB  - Monoclonal immunoglobulin (Ig) deposition diseases are characterized by 
      deposition in tissues of excessive amounts of the Ig, compromising organ 
      functions. Light chain deposition disease (LCDD) and AL amyloidosis are the 
      commonest [Buxbaum 1992]. LCDD is usually characterized by rapidly progressive 
      renal failure with glomerular and tubular deposits of Ig fragments mostly 
      composed by kappa light chain. Monoclonal Ig production can also be observed 
      associated with various symptoms, that, taken together, have been described as 
      the Crow-Fukase syndrome or POEMS syndrome. It associates polyneuropathy, 
      organomegaly, endocrinopathy, monoclonal Ig, and skin changes. In POEMS syndrome, 
      renal abnormalities are rare and are reported as a moderate renal insufficiency 
      with mild proteinuria or acute functional renal insufficiency leading in some 
      cases to end-stage renal failure [Fukatsu et al. 1991]. Although a monoclonal Ig 
      is produced, no Ig deposit disease had been described in POEMS syndrome except a 
      case of AL amyloidosis [Toyokuni et al. 1992]. Here, to our knowledge, we report 
      the first case of an LCDD associated with a POEMS syndrome. Although an 
      autologous bone marrow graft was realized, the monoclonal component reappeared 
      and was responsible for end-stage renal disease, cachexia and death.
FAU - Lambotte, O
AU  - Lambotte O
AD  - Service de Nephrologie, CHU du Kremlin Bicetre, France.
FAU - Durrbach, A
AU  - Durrbach A
FAU - Ammor, M
AU  - Ammor M
FAU - Paradis, V
AU  - Paradis V
FAU - Djeffal, R
AU  - Djeffal R
FAU - Machover, D
AU  - Machover D
FAU - Charpentier, B
AU  - Charpentier B
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Review
PL  - Germany
TA  - Clin Nephrol
JT  - Clinical nephrology
JID - 0364441
RN  - 0 (Immunoglobulin kappa-Chains)
SB  - IM
MH  - Adult
MH  - Humans
MH  - Hypergammaglobulinemia/*complications/epidemiology
MH  - Immunoglobulin kappa-Chains/*analysis
MH  - Kidney/pathology
MH  - Male
MH  - POEMS Syndrome/*complications/epidemiology
RF  - 13
EDAT- 2001/07/04 10:00
MHDA- 2001/11/03 10:01
CRDT- 2001/07/04 10:00
PHST- 2001/07/04 10:00 [pubmed]
PHST- 2001/11/03 10:01 [medline]
PHST- 2001/07/04 10:00 [entrez]
PST - ppublish
SO  - Clin Nephrol. 2001 Jun;55(6):482-6.