Skip to main content
Postgraduate Medical Journal logoLink to Postgraduate Medical Journal
. 1988 Oct;64(756):804–808. doi: 10.1136/pgmj.64.756.804

Light chain deposition disease presenting with hepatomegaly: an association with amyloid-like fibrils.

G Pelletier 1, M Fabre 1, P Attali 1, A Ladouch-Badre 1, O Ink 1, E Martin 1, J P Etienne 1
PMCID: PMC2428999  PMID: 3151378

Abstract

We report an unusual case of lambda light chain deposits without overt plasma cell dyscrasia. The clinical presentation was hepatomegaly without biochemical sign of renal involvement. Portal hypertension, spontaneous rupture of the spleen and fracture of the 12th thoracic vertebra occurred during the course of the disease. Ultrastructural studies showed that lambda light chain deposits were associated with amyloid-like deposits. This case suggests that light chain deposition disease and amyloidosis could be two expressions of the same disease.

Full text

PDF
804

Images in this article

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Alpers C. E., Hopper J., Jr, Biava C. G. Light-chain glomerulopathy with amyloid-like deposits. Hum Pathol. 1984 May;15(5):444–448. doi: 10.1016/s0046-8177(84)80078-7. [DOI] [PubMed] [Google Scholar]
  2. Droz D., Noel L. H., Carnot F., Degos F., Ganeval D., Grunfeld J. P. Liver involvement in nonamyloid light chain deposits disease. Lab Invest. 1984 Jun;50(6):683–689. [PubMed] [Google Scholar]
  3. Glenner G. G. Amyloid deposits and amyloidosis. The beta-fibrilloses (first of two parts). N Engl J Med. 1980 Jun 5;302(23):1283–1292. doi: 10.1056/NEJM198006053022305. [DOI] [PubMed] [Google Scholar]
  4. Hoffman-Guilaine C., Nochy D., Tricottet V., Mallet L., Bariety J., Camilleri J. P. La maladie des dépôts de chaînes légères: une entité anatomopathologique. Ann Pathol. 1984 Apr-May;4(2):105–113. [PubMed] [Google Scholar]
  5. Kirkpatrick C. J., Curry A., Galle J., Melzner I. Systemic kappa light chain deposition and amyloidosis in multiple myeloma: novel morphological observations. Histopathology. 1986 Oct;10(10):1065–1076. doi: 10.1111/j.1365-2559.1986.tb02543.x. [DOI] [PubMed] [Google Scholar]
  6. Kyle R. A., Bayrd E. D. Amyloidosis: review of 236 cases. Medicine (Baltimore) 1975 Jul;54(4):271–299. doi: 10.1097/00005792-197507000-00001. [DOI] [PubMed] [Google Scholar]
  7. LEVINE R. A. Amyloid disease of the liver. Correlation of clinical, functional and morphologic features in forty-seven patients. Am J Med. 1962 Sep;33:349–357. doi: 10.1016/0002-9343(62)90231-0. [DOI] [PubMed] [Google Scholar]
  8. Mignon F., Morel-Maroger L., Cerf M., Preud'homme J. L., Richet G. Dépôts hépatiques et rénaux de chaînes légères kappa révélateurs d'une dysglobulinémie. Nephrologie. 1980;1(4):167–170. [PubMed] [Google Scholar]
  9. Randall R. E., Williamson W. C., Jr, Mullinax F., Tung M. Y., Still W. J. Manifestations of systemic light chain deposition. Am J Med. 1976 Feb;60(2):293–299. doi: 10.1016/0002-9343(76)90440-x. [DOI] [PubMed] [Google Scholar]

Articles from Postgraduate Medical Journal are provided here courtesy of BMJ Publishing Group

RESOURCES