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


METHODS
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.


CONCLUSIONS
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.
Foguem
C
C
Service de médecine interne, hôpital Jean-Minjoz, CHU de Besançon, boulevard Fleming, 25030 Besançon cedex, France. cfoguem@chu-besancon.fr
Kantelip
B
B
Deconinck
E
E
Hafsaoui
C
C
Méaux-Ruault
N
N
Gil
H
H
Magy-Bertrand
N
N
Dupond
J-L
JL
D001706:Biopsy; D003937:Diagnosis, Differential; D004576:Electromyography; D005260:Female; D006801:Humans; D006984:Hypertrophy; D007145:Immunoglobulin kappa-Chains; D008558:Melphalan / Q000008:administration & dosage*; D010265:Paraproteinemias / Q000150:complications / Q000175:diagnosis; D010523:Peripheral Nervous System Diseases / Q000150:complications / Q000175:diagnosis; D012469:Salivary Glands / Q000473:pathology; D012796:Sialography; D012859:Sjogren's Syndrome / Q000175:diagnosis*; D033581:Stem Cell Transplantation; D014057:Tomography, X-Ray Computed; D014182:Transplantation, Autologous
D007145:Immunoglobulin kappa-Chains; D008558:Melphalan