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

AbstractINTRODUCTION:
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.
AuthorsC Foguem, B Kantelip, E Deconinck, C Hafsaoui, N Méaux-Ruault, H Gil, N Magy-Bertrand, J-L Dupond
JournalLa Revue de medecine interne (Rev Med Interne) Vol. 30 Issue 1 Pg. 49-52 (Jan 2009) ISSN: 0248-8663 [Print] France
Vernacular TitleMaladie de Randall, simulant une maladie de Gougerot-Sjögren, traitée avec succès par chimiothérapie et autogreffe de moelle osseuse.
PMID18801600 (Publication Type: Case Reports, English Abstract, Journal Article)
Chemical References
  • Immunoglobulin kappa-Chains
  • Melphalan
Topics
  • Biopsy
  • Diagnosis, Differential
  • Electromyography
  • Female
  • Humans
  • Hypertrophy
  • Immunoglobulin kappa-Chains
  • Melphalan (administration & dosage)
  • Paraproteinemias (complications, diagnosis)
  • Peripheral Nervous System Diseases (complications, diagnosis)
  • Salivary Glands (pathology)
  • Sialography
  • Sjogren's Syndrome (diagnosis)
  • Stem Cell Transplantation
  • Tomography, X-Ray Computed
  • Transplantation, Autologous

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