Light-Chain Deposition Disease Presenting with Spontaneous Splenic Rupture

Luís Pontes dos Santos; Joana Couto; Miguel Romano; Raquel López

doi:10.12890/2018_001010

Light-Chain Deposition Disease Presenting with Spontaneous Splenic Rupture

Luís Pontes dos Santos Serviço de Medicina 1, ULSAM, Viana do Castelo, Portugal http://orcid.org/0000-0001-5821-8238
Joana Couto Serviço de Medicina 1, ULSAM, Viana do Castelo, Portugal http://orcid.org/0000-0003-4024-415X
Miguel Romano Serviço de Medicina 1, ULSAM, Viana do Castelo, Portugal http://orcid.org/0000-0003-2821-867X
Raquel López Serviço de Medicina 1, ULSAM, Viana do Castelo, Portugal http://orcid.org/0000-0001-5138-2967

DOI: https://doi.org/10.12890/2018_001010

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.

References

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Published: 2018-12-19

Issue: Vol 5 No 12 (view)

Section: Articles

How to cite:

Pontes dos Santos, L., Couto, J., Romano, M., & López, R. (2018). Light-Chain Deposition Disease Presenting with Spontaneous Splenic Rupture. European Journal of Case Reports in Internal Medicine, 5(12). https://doi.org/https://doi.org/10.12890/2018_001010