PMID- 12118397
OWN - NLM
STAT- MEDLINE
DCOM- 20021002
LR  - 20180323
IS  - 0270-9295 (Print)
IS  - 0270-9295 (Linking)
VI  - 22
IP  - 4
DP  - 2002 Jul
TI  - Kidney and liver involvement in monoclonal light chain disorders.
PG  - 319-30
AB  - Monoclonal light chains (LCs) are responsible for a wide spectrum of renal and 
      hepatic diseases, that above all include amyloid light-chain (AL) amyloidosis and 
      light chain deposition disease (LCDD). Amyloid deposits stain for Congo red on 
      light microscopy and have fibrillar aspect on electron microscopy, whereas 
      deposits in LCDD are positive using monotypic LCs on immunofluorescence and have 
      a granular aspect on electron microscopy. Sometimes fibrillar and granular 
      deposits are observed in the same organ or in different organs of the same 
      patient. Kidney and liver involvement is a frequent finding, both in primary 
      amyloidosis (AL amyloidosis) or in LCDD. Renal manifestations include 
      proteinuria, nephrotic syndrome, and progressive renal failure. End-stage renal 
      disease requiring dialysis is observed in about 20% of patients with AL 
      amyloidosis and in 70% of patients with LCDD. The mean survival time is about 12 
      to 18 months in AL amyloidosis and 34 months in LCDD. The most important 
      prognostic factor is severe cardiac involvement, which reduces the mean survival 
      to only 6 months. Hepatic manifestations include hepatomegaly, portal 
      hypertension, ascites, intrahepatic cholostatic jaundice, and hepatic 
      insufficiency. The mean survival of patients with liver damage is 14 months, but 
      it is reduced to 5 months in patients with cholostatic jaundice. Contemporary 
      kidney and liver involvement is usually observed on histologic examination, less 
      frequently as clinical manifestation. No specific treatment exists for AL 
      amyloidosis and LCDD, and the prognosis remains severe. The aim of treatment is 
      to suppress proliferation of the abnormal clone of plasma cells and remove tissue 
      deposits. The regimens, including melphalan-prednisone (MP) or 
      vincristine-doxorubicin-dexamethasone (VAD), are used both in AL amyloidosis or 
      in LCDD with some effectiveness. New approaches, especially the use of 
      4'-iodo-4'deoxydoxorubicin, could achieve better results. Dialysis seems to not 
      worsen the outcome in both diseases because survival of patients on dialysis is 
      not different from that of patients not reaching uremia. Also, kidney and liver 
      transplantation is effective, though amyloidosis or LCDD may occur in 
      transplanted organs. The most interesting therapeutic approach is 
      autologous-blood stem-cell transplantation, which may produce a complete 
      remission of the plasma-cell dyscrasia and a substantial improvement of clinical 
      manifestations related to LC deposits.
CI  - Copyright 2002, Elsevier Science (USA). All rights reserved.
FAU - Pozzi, Claudio
AU  - Pozzi C
AD  - Division of Nephrology and Dialysis, A. Manzoni Hospital, Lecco, Italy. 
      nefrologia@ospedale.lecco.it
FAU - Locatelli, Francesco
AU  - Locatelli F
LA  - eng
PT  - Journal Article
PT  - Review
PL  - United States
TA  - Semin Nephrol
JT  - Seminars in nephrology
JID - 8110298
RN  - 0 (Immunoglobulin Light Chains)
SB  - IM
MH  - Amyloidosis/*complications/therapy
MH  - Humans
MH  - Immunoglobulin Light Chains/*metabolism
MH  - Kidney Diseases/*etiology/metabolism/therapy
MH  - Liver Diseases/*etiology/metabolism/therapy
MH  - Prognosis
RF  - 115
EDAT- 2002/07/16 10:00
MHDA- 2002/10/03 04:00
CRDT- 2002/07/16 10:00
PHST- 2002/07/16 10:00 [pubmed]
PHST- 2002/10/03 04:00 [medline]
PHST- 2002/07/16 10:00 [entrez]
AID - S0270-9295(02)70036-3 [pii]
PST - ppublish
SO  - Semin Nephrol. 2002 Jul;22(4):319-30.