Light chain deposition disease of the liver without renal involvement in a patient with multiple myeloma related to liver failure and rapid fatal outcome.
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.
Michopoulos
Spyros
S
Gastroenterology Unit, Alexandra University Hospital, Athens, Greece.
Petraki
Kalliopi
K
Petraki
Constantina
C
Dimopoulos
Meletios-Athanasios
MA
D000328:Adult; D017809:Fatal Outcome; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000378:metabolism*; D008099:Liver / Q000378:metabolism / Q000473:pathology; D008107:Liver Diseases / Q000150:complications* / Q000378:metabolism* / Q000473:pathology; D017093:Liver Failure / Q000209:etiology*; D008297:Male; D009101:Multiple Myeloma / Q000150:complications*; D013997:Time Factors
D007147:Immunoglobulin Light Chains