[Kappa light chain deposition disease, presenting as Sjogren's syndrome, successfully treated by high-dose melphalan and autologous blood stem transplantation]
Fiche publication
Date publication
janvier 2009
Auteurs
Membres identifiés du Cancéropôle Est :
Pr DECONINCK Eric
Tous les auteurs :
Foguem C, Kantelip B, Deconinck E, Hafsaoui C, Meaux-Ruault N, Gil H, Magy-Bertrand N, Dupond JL
Lien Pubmed
Résumé
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 Sjogren'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.
Référence
Rev Med Interne. 2009 Jan;30(1):49-52