Light-Chain Deposition Disease Presenting with Spontaneous Splenic Rupture

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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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
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

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