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Recovery from LCDD-associated Severe Liver Cholestasis: a Case Report and Literature Review

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Benoit Brillandl,2, Johnny Sayeghl,2, Anne Crouel,3, Frank Bridoux4, Jean-François Subral,2, Jean-François Augustol,2

1) LUNAM Université, Angers,
2) CHU Angers, Service de Néphrologie-Dialyse-Transplantation,
3) CHU Angers, Département de Pathologie Cellulaire et Tissulaire, Angers
4) Département de Néphrologie et Transplantation, CHU Poitiers, Poitiers, France

DOI: http://dx.doi.org/10.15403/jgld.2014.1121.251.lcd


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.

Key words: light chain deposition disease – myeloma – liver – cholestatic hepatitis.

Abbreviations: AKI: Acute kidney injury; ALP: alkaline phosphatase; ALT: alanine aminotransferase; AST: aspartate aminotransferase; CMV: Cytomegalovirus; EBV: Epstein–Barr virus; GGT: gamma-glutamyl transferase; HSV: Herpes simplex virus; LC: light chain; LCDD: Light chain deposition disease; MIDD: Monoclonal immunoglobulin deposition disease; MM: Multiple myeloma.