Light-Chain Deposition Disease Presenting with Spontaneous Splenic Rupture

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.

Spontaneous splenic rupture is a rare presentation of a paraproteinaemia.Light-chain deposition disease is a rare disease with non-fibrillar deposition of monoclonal light-chain immunoglobulins most frequently affecting the kidney.Light-chain deposition disease should be suspected in case of a monoclonal plasma cell disorder with Congo-red negative deposits on histology.