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

• Published in: La revue de medecine interne Vol. 30; no. 1; pp. 49 - 52
• Main Authors: Foguem, C, Kantelip, B, Deconinck, E, Hafsaoui, C, Méaux-Ruault, N, Gil, H, Magy-Bertrand, N, Dupond, J-L
• Format: Journal Article
• Language: French
• Published: France Elsevier 01.01.2009
• Subjects: Biopsy; Diagnosis, Differential; Electromyography; Female; Humans; Hypertrophy; Immunoglobulin kappa-Chains; Immunology; Life Sciences; Melphalan; Melphalan - administration & dosage; Paraproteinemias; Paraproteinemias - complications; Paraproteinemias - diagnosis; Peripheral Nervous System Diseases; Peripheral Nervous System Diseases - complications; Peripheral Nervous System Diseases - diagnosis; Salivary Glands; Salivary Glands - pathology; Sialography; Sjogren's Syndrome; Sjogren's Syndrome - diagnosis; Stem Cell Transplantation; Tomography, X-Ray Computed; Transplantation, Autologous
• ISSN: 0248-8663; 1768-3122
• DOI: 10.1016/j.revmed.2008.08.003

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. 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. 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.