Light chain deposition disease affecting the gastrointestinal tract in the setting of post-living donor kidney transplantation.

International Journal of Hematology
V H Jimenez-ZepedaSuzanne Trudel

Abstract

Light chain deposition disease (LCDD) is an uncommon, clonal plasma cell proliferative disorder, in which monoclonal immunoglobulin light chains deposit in various tissues, resulting in organ dysfunction. Gastrointestinal (GI) involvement has been described in both primary and secondary amyloidosis, but has rarely been reported in LCDD, and only as an incidental finding. We report a case of LCDD in living related kidney transplant recipient presenting with severe GI dysmotility, weight loss and progressive allograft dysfunction. A diagnosis of LCDD was based on the kidney biopsy findings in the failing renal allograft, along with the presence of excess serum free kappa light chains and abnormal kappa:lambda ratio. Subsequent review of GI biopsies confirmed kappa light chain immunoglobulin deposition within the stomach. Further investigation suggested additional hepatic and cardiac involvement. The patient went on to receive bortezomib, achieving a biochemical response and stabilization of his advanced renal dysfunction; however, bortezomib was discontinued due to toxicity. The patient was subsequently treated with lenalidomide and dexamethasone, which were better tolerated. Further biochemical response and resolution of the GI ...Continue Reading

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Sep 6, 2014·Ultrastructural Pathology·Kuang-Yu JenLinda D Ferrell
May 1, 2019·The Cochrane Database of Systematic Reviews·Daniel Molano FrancoJavier Zamora
Aug 28, 2021·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Molly Delk Plummer, Fredric Regenstein

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