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.

References

  • Sayed R, Wechalekar A, Gilbertson J, Bass P, Mahmood S, Sachchithanantham S, et al. Natural history and outcome of light chain deposition disease. Blood 2015;126:2805–2810.

  • Said S, Cooper C, Nwosu A, Bilbao J, Hernandez G. Hypertension, renal failure, and edema in a 38-year-old man: light chain deposition disease; a case report and review of the literature. J Nephropathol 2014;3:63–68.

  • Renzulli P, Schoepfer A, Mueller E, Candinas D. Atraumatic splenic rupture in amyloidosis. Amyloid 2009;16:47–53.

  • Lee G, Duan-Porter W, Metjian A. Acquired, non-amyloid related factor X deficiency: review of the literature. Haemophilia 2012;18:655–663.

  • Wada Y, Iyoda M, Saito T, Arai-Nunota N, Iseri K, Tomita E, et al. Light-chain deposition disease successfully treated with bortezomib in an elderly patient: a case report and review of the literature. Intern Med 2015;54:2893–2898.
  • 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