title: Recovery from LCDD-associated Severe Liver Cholestasis: a Case Report and Literature Review.
abstract: Light chain deposition disease (LCDD) is a rare multisystemic disorder associated with plasma cell proliferation. It mainly affects the kidney, but liver and heart involvement may occur, sometimes mimicking the picture of systemic amyloidosis. Liver disease in LCDD is usually asymptomatic and exceptionally manifests with severe cholestatic hepatitis. We report the case of a 66-year-old female with κ-LCDD and cast nephropathy in the setting of symptomatic multiple myeloma who, after a first cycle of bortezomib-dexamethasone chemotherapy, developed severe and rapidly worsening intrahepatic cholestasis secondary to liver κ-light chain deposition. Intrahepatic cholestasis was attributed to LCDD on the basis of the liver histology and exclusion of possible diagnoses. Chemotherapy was maintained and resulted in progressive resolution of cholestasis. We report here an uncommon presentation of LCDD, with prominent liver involvement that fully recovered with bortezomib-based chemotherapy, and briefly review the relevant literature.
authors.affiliation: LUNAM Université, Angers;CHU Angers, Service de Néphrologie-Dialyse-Transplantation, Poitiers, France.
authors.forename: Benoit
authors.initials: B
authors.lastname: Brilland
authors.affiliation: LUNAM Université, Angers;CHU Angers, Service de Néphrologie-Dialyse-Transplantation, Poitiers, France.
authors.forename: Johnny
authors.initials: J
authors.lastname: Sayegh
authors.affiliation: LUNAM Université, Angers;CHU Angers, Département de Pathologie Cellulaire et Tissulaire, Angers, France.
authors.forename: Anne
authors.initials: A
authors.lastname: Croue
authors.affiliation: Département de Néphrologie et Transplantation, CHU Poitiers, Poitiers, France.
authors.forename: Frank
authors.initials: F
authors.lastname: Bridoux
authors.affiliation: LUNAM Université, Angers;CHU Angers, Service de Néphrologie-Dialyse-Transplantation, Poitiers, France.
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Subra
authors.affiliation: LUNAM Université, Angers;CHU Angers, Service de Néphrologie-Dialyse-Transplantation, Poitiers, France. jfaugusto@chu-angers.fr.
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Augusto
mesh_terms: D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001706:Biopsy; D000069286:Bortezomib / Q000008:administration & dosage; D002780:Cholestasis, Intrahepatic / Q000175:diagnosis / Q000209:etiology* / Q000276:immunology*; D003907:Dexamethasone / Q000008:administration & dosage; D005260:Female; D005455:Fluorescent Antibody Technique; D006801:Humans; D007145:Immunoglobulin kappa-Chains / Q000276:immunology*; D008099:Liver / Q000276:immunology* / Q000473:pathology; D009101:Multiple Myeloma / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology; D016896:Treatment Outcome
chemical_list: D007145:Immunoglobulin kappa-Chains; D000069286:Bortezomib; D003907:Dexamethasone