Light-Chain Deposition Disease Presenting with Spontaneous Splenic Rupture

Keywords

Light-chain deposition disease, paraproteinemia, splenic rupture

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.

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    Published: 2018-12-19
    Issue: Vol 5 No 12 (view)

    How to cite:
    1.
    Pontes dos Santos L, Couto J, Romano M, López R. Light-Chain Deposition Disease Presenting with Spontaneous Splenic Rupture. EJCRIM 2018;5 doi:10.12890/2018_001010.

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