[Kappa light chain deposition disease, presenting as Sjögren's syndrome, successfully treated by high-dose melphalan and autologous blood stem transplantation] - Inserm - Institut national de la santé et de la recherche médicale Access content directly
Journal Articles La Revue de Médecine Interne Year : 2009

[Kappa light chain deposition disease, presenting as Sjögren's syndrome, successfully treated by high-dose melphalan and autologous blood stem transplantation]

Abstract

INTRODUCTION: Light chain deposition disease is a systemic disorder characterised by tissue deposition of monoclonal immunoglobulin light chains without tinctorial properties. It has been exceptionally reported with salivary involvement mimicking Sjögren's syndrome and peripheral neuropathy. CASE REPORT: We report a case of light chain deposition disease associated with plasma cell dyscrasia presenting as sicca syndrome with salivary glands hypertrophy and polyneuropathy successfully treated by high dose melphalan and autologous blood stem transplantation. CONCLUSION: Light chain deposition disease should be recognized as an aetiology of sicca syndrome and peripheral neuropathy. Further studies should assess the prevalence of sicca syndrome in light chain deposition disease and better characterise the neurological manifestations.

Domains

Immunology

Dates and versions

inserm-00484589 , version 1 (18-05-2010)

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Cite

C. Foguem, Bernadette Kantelip, Eric Deconinck, Chafika Hafsaoui, Nadine Méaux-Ruault, et al.. [Kappa light chain deposition disease, presenting as Sjögren's syndrome, successfully treated by high-dose melphalan and autologous blood stem transplantation]. La Revue de Médecine Interne, 2009, 30 (1), pp.49-52. ⟨10.1016/j.revmed.2008.08.003⟩. ⟨inserm-00484589⟩

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