[A case of acute renal and liver dysfunction with light chain deposition disease]

Abstract

A 62 year old woman was referred to our hospital because of acute renal and liver dysfunction. Prior to admission, she had already been started on hemodyalysis filtration(HDF). She showed facial edema and lumbar pain caused by an Ll compressive fracture. Laboratory examinations revealed hypercalcemia (13.2 mg/dL), hyperammonemia (297 microg/dL) and her serum creatinine, blood urea nitrogen and total bilirubin levels were 3.9 mg/dL, 37.4 mg/dL and 3.2 mg/dL, respectively. Among the components of immunoglobulin, IgA was increased, while IgG and IgM were decreased. Serum immunoelectrophoresis revealed the presence of the IgA kappa type of M component. Punched out lesions were noted on her head radiography. Severe plasmacytosis (60-70 % of total cells) were observed by a bone marrow aspiration test, indicating the diagnosis of multiple myeloma. Steroid pulse therapy was started with dexamethasone (40 mg/day, 3 days), and plasma exchange was performed 8 times with continuous HDF. These treatments failed to control hemodynamics and she died of disseminated intravascular coagulation (DIC). Autopsy demonstrated amyloid-like depositions in perisinusoidal space in the liver. In the kidney, there were nodular lesions in the glomeruli, and depositions in the basement membrane of the uriniferous tubuli. Congo red staining of these organs for amyloid yielded negative results. Immunohistochemical staining gave positive results for IgA and kappa. Electron microscopy revealed granular electron deposits in the glomeruli and tubular basement membrane as well. Taken altogether, the diagnosis of the patient could be light chain deposition disease (LCDD).