title: 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.
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.
authors.affiliation: Medical Center, Department of Hematology, Oncology and Immunology, University of Tübingen, Otfried-Müller-Strasse 10, 72076, Tübingen, Germany. katja.weisel@med.uni-tuebingen.de.
authors.forename: Katja C
authors.initials: KC
authors.lastname: Weisel
authors.affiliation: Medical Center, Department of Hematology, Oncology and Immunology, University of Tübingen, Otfried-Müller-Strasse 10, 72076, Tübingen, Germany.
authors.forename: Michael
authors.initials: M
authors.lastname: Böckeler
authors.affiliation: Institute for Pathology, Freiburg, Germany.
authors.forename: Leonardo
authors.initials: L
authors.lastname: Bianchi
authors.affiliation: Institute for Pathology, Freiburg, Germany.
authors.forename: Luigi M
authors.initials: LM
authors.lastname: Terracciano
authors.affiliation: Medical Center, Department of Hematology, Oncology and Immunology, University of Tübingen, Otfried-Müller-Strasse 10, 72076, Tübingen, Germany.
authors.forename: Frank
authors.initials: F
authors.lastname: Mayer
authors.affiliation: Medical Center, Department of Hematology, Oncology and Immunology, University of Tübingen, Otfried-Müller-Strasse 10, 72076, Tübingen, Germany.
authors.forename: Lothar
authors.initials: L
authors.lastname: Kanz
mesh_terms: D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects*; D002761:Cholangitis / Q000139:chemically induced / Q000209:etiology* / Q000473:pathology; D006499:Hepatic Artery / Q000473:pathology*; D006801:Humans; D007147:Immunoglobulin Light Chains; D007511:Ischemia; D000077269:Lenalidomide; D008297:Male; D008558:Melphalan / Q000009:adverse effects; D009101:Multiple Myeloma / Q000150:complications* / Q000188:drug therapy; D010265:Paraproteinemias / Q000150:complications; D011241:Prednisone / Q000009:adverse effects; D013792:Thalidomide / Q000009:adverse effects / Q000031:analogs & derivatives
chemical_list: D007147:Immunoglobulin Light Chains; D013792:Thalidomide; D000077269:Lenalidomide; D008558:Melphalan; D011241:Prednisone