Development of rapid light-chain deposition disease in hepatic arteries with severe ischemic cholangitis in a multiple myeloma patient treated with melphalan, prednisone and lenalidomide |
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Authors: | Katja C Weisel Michael Böckeler Leonardo Bianchi Luigi M Terracciano Frank Mayer Lothar Kanz |
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Institution: | (1) Medical Center, Department of Hematology, Oncology and Immunology, University of Tübingen, Otfried-Müller-Strasse 10, 72076 Tübingen, Germany;(2) Institute for Pathology, Freiburg, Germany |
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Abstract: | Light-chain deposition disease (LCDD) is a multisystemic disorder associated with plasma cell dyscrasias and multiple myeloma.
It is histologically characterized by the deposition of a homogeneous, in electron microscopy granular, slightly eosinophilic
material showing positivity usually for kappa light chains. In contrast to AL-amyloidosis, the material is negative for Congo
red. LCDD mainly involves the kidneys as the predominant organ manifestation resulting in a nephrotic syndrome. However, involvement
of other tissues such as liver and heart have been described. Here we report a case of severe ischemic cholangitis in a patient
with multiple myeloma receiving chemotherapy with melphalan, prednisone, and lenalidomide. Histopathological analysis revealed
LCDD of the hepatic arteries as the underlying cause. This is to our knowledge the first case of LCDD of terminal liver arteries
as a cause of intrahepatic ischemic cholangitis. |
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Keywords: | LCDD Lenalidomide Multiple myeloma Ischemic cholangitis Hepatotoxicity |
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