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.
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.
Weisel
Katja C
KC
Medical Center, Department of Hematology, Oncology and Immunology, University of Tübingen, Otfried-Müller-Strasse 10, 72076, Tübingen, Germany. katja.weisel@med.uni-tuebingen.de.
Böckeler
Michael
M
Medical Center, Department of Hematology, Oncology and Immunology, University of Tübingen, Otfried-Müller-Strasse 10, 72076, Tübingen, Germany.
Bianchi
Leonardo
L
Institute for Pathology, Freiburg, Germany.
Terracciano
Luigi M
LM
Institute for Pathology, Freiburg, Germany.
Mayer
Frank
F
Medical Center, Department of Hematology, Oncology and Immunology, University of Tübingen, Otfried-Müller-Strasse 10, 72076, Tübingen, Germany.
Kanz
Lothar
L
Medical Center, Department of Hematology, Oncology and Immunology, University of Tübingen, Otfried-Müller-Strasse 10, 72076, Tübingen, Germany.
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
D007147:Immunoglobulin Light Chains; D013792:Thalidomide; D000077269:Lenalidomide; D008558:Melphalan; D011241:Prednisone