PMID- 11991600
OWN - NLM
STAT- MEDLINE
DCOM- 20020523
LR  - 20190721
IS  - 0163-2116 (Print)
IS  - 0163-2116 (Linking)
VI  - 47
IP  - 4
DP  - 2002 Apr
TI  - Light chain deposition disease of the liver without renal involvement in a 
      patient with multiple myeloma related to liver failure and rapid fatal outcome.
PG  - 730-4
AB  - We describe a 36-year-old man with advanced multiple myeloma (Salmon and Durie 
      stage III) who developed jaundice and severe cholestasis after a first cure with 
      systemic chemotherapy of vincristine, doxorubicin, and oral dexamethasone (VAD). 
      Serology for hepatitis A, B, and C and for CMV was negative. A liver ultrasound 
      and CT scan showed mild hepatomegaly without evidence of extrahepatic or 
      intrahepatic biliary tree dilatation. A percutaneous liver biopsy revealed 
      perisinusoidal deposits of an abundant slightly eosinophilic, PAS-positive 
      amorphous substance. Immunohistochemistry showed positivity for kappa-light 
      chains and was negative for lambda-light chains, for IgA, IgG, IgM, and IgD 
      immunoglobulins as well as for AA and AL proteins and for amyloid P component. A 
      diagnosis of light chain deposition disease (LCDD) of the liver was made. The 
      patient developed rapid deterioration of liver function, leading to a multisystem 
      dysfunction and death. The occurrence of LCDD in multiple myeloma is close to 5% 
      and myeloma is the underlying disease in two thirds of patients with LCDD. The 
      kidneys are involved in almost all cases of LCDD and renal dysfunction usually 
      reveals the disease. Only three patients with LCDD of the liver without overt 
      renal involvement have been reported so far. This is the first observation of 
      LCDD presenting with jaundice and severe cholestasis shortly after the diagnosis 
      of high tumor mass myeloma, without overt renal involvement, leading rapidly to 
      the patient's death.
FAU - Michopoulos, Spyros
AU  - Michopoulos S
AD  - Gastroenterology Unit, Alexandra University Hospital, Athens, Greece.
FAU - Petraki, Kalliopi
AU  - Petraki K
FAU - Petraki, Constantina
AU  - Petraki C
FAU - Dimopoulos, Meletios-Athanasios
AU  - Dimopoulos MA
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - United States
TA  - Dig Dis Sci
JT  - Digestive diseases and sciences
JID - 7902782
RN  - 0 (Immunoglobulin Light Chains)
SB  - IM
MH  - Adult
MH  - Fatal Outcome
MH  - Humans
MH  - Immunoglobulin Light Chains/*metabolism
MH  - Liver/metabolism/pathology
MH  - Liver Diseases/*complications/*metabolism/pathology
MH  - Liver Failure/*etiology
MH  - Male
MH  - Multiple Myeloma/*complications
MH  - Time Factors
EDAT- 2002/05/07 10:00
MHDA- 2002/05/25 10:01
CRDT- 2002/05/07 10:00
PHST- 2002/05/07 10:00 [pubmed]
PHST- 2002/05/25 10:01 [medline]
PHST- 2002/05/07 10:00 [entrez]
AID - 10.1023/a:1014773512707 [doi]
PST - ppublish
SO  - Dig Dis Sci. 2002 Apr;47(4):730-4. doi: 10.1023/a:1014773512707.