Abstract：
: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.
journal_name
Dig Dis Scijournal_title
Digestive diseases and sciencesauthors
Michopoulos S,Petraki K,Petraki C,Dimopoulos MAdoi
10.1023/a:1014773512707subject
Has Abstractpub_date
2002-04-01 00:00:00pages
730-4issue
4eissn
0163-2116issn
1573-2568journal_volume
47pub_type
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journal_title：Digestive diseases and sciences
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pub_type： 临床试验,杂志文章
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pub_type： 杂志文章
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journal_title：Digestive diseases and sciences
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journal_title：Digestive diseases and sciences
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journal_title：Digestive diseases and sciences
pub_type： 临床试验,杂志文章,随机对照试验
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