DOI: 10.12890/2018_001010

Light-Chain Deposition Disease Presenting with Spontaneous Splenic Rupture

Authors

  • Luís Pontes dos Santos Serviço de Medicina 1, ULSAM, Viana do Castelo, Portugal
  • Joana Couto Serviço de Medicina 1, ULSAM, Viana do Castelo, Portugal
  • Miguel Romano Serviço de Medicina 1, ULSAM, Viana do Castelo, Portugal
  • Raquel López Serviço de Medicina 1, ULSAM, Viana do Castelo, Portugal

lmp_santos@sapo.pt

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 (2018)

How to Cite

1.
Pontes dos Santos L, Couto J, Romano M, López R. Light-Chain Deposition Disease Presenting with Spontaneous Splenic Rupture. EJCRIM [Internet]. 2018 Dec. 19 [cited 2026 Nov. 3];5(12). Available from: https://ejcrim.com/index.php/ejcrim/article/view/1010

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