Pontes dos Santos, Luís and Couto, Joana and Romano, Miguel and López, Raquel (2018) Light-Chain Deposition Disease Presenting with Spontaneous Splenic Rupture. European Journal of Case Reports in Internal Medicine, 5 (12). ISSN 2284-2594
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Abstract
Light-chain deposition disease is a rare paraproteinaemia characterized by deposition of monoclonal light-chain immunoglobulins with a non-amyloid structure, most frequently affecting the kidney. The authors present the case of a 58-year-old man admitted due to haemorrhagic shock caused by spontaneous splenic rupture. Investigation showed coagulopathy, homogeneous hepatomegaly with elevated cholestasis enzymes but normal bilirubinaemia, and a nephrotic syndrome with monoclonal kappa light chains. Liver and bone marrow biopsies revealed light-chain deposition disease. Splenic rupture as the presentation of a paraproteinaemia is rare. The diagnosis of light-chain deposition disease should be considered in case of a monoclonal plasma cell disorder with Congo-red negative deposits on histology.
Item Type: | Article |
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Uncontrolled Keywords: | Light-chain deposition disease, paraproteinemia, splenic rupture |
Subjects: | 600 Tecnologia - Scienze applicate > 610 Medicina e salute (Classificare qui la tecnologia dei servizi medici) > 616 Malattie (classificare qui la Clinica medica, la medicina basata sull'evidenza, la Medicina interna, la Medicina sperimentale) |
Depositing User: | Greta Di Blasio |
Date Deposited: | 10 Jun 2019 16:12 |
Last Modified: | 10 Jun 2019 16:12 |
URI: | http://eprints.bice.rm.cnr.it/id/eprint/18837 |
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