Light chain disease
Keywords:
ascites, jaundice, hepatomegaly, light chain disease, plasma cell dyscrasiaAbstract
The authors describe an unusual case of light chain deposition disease in which the clinicai presentation was hepatomegaly, ascites and jaundice, without biochemical evidence of renal involvement.
Light chain disease of the kidney, kappa class, is frequent, and deposition of light chains rarely occur in other organs as the first manifestation of this plasma cell dyscrasia.
Downloads
References
Glenner GG. Amyloid deposits and amyloidosis. The b-fibrilloses. N Engl J Med 1980: 302: l 283-1292.1333-1343.
Shustik C. Bergsagel DE and Pruzanski W.:k and I light chain disease: Survival rates and clinical manifestations. Blood 1976: 48: 41-51.
Linder J. Crocker BP. Vollmer RT and Shelburne J. Systemic kappa light-chain deposition: An ultrastructural and immunohistochemical study. Am J Surg Pathol 1983: 7: 85-93.
Randall RE. Williamson WC. Mullinax F. Tung MY. and Still WJS. Manifestations of systemic light chain deposition. Am J Med 1976: 60: 293-299.
Preud'Homme JL. Aucouturier P. Touchard G, Striker L, Khamlichi AA. Rocca A. Denoroy L. and Cogné M. Monoclonal immuno globulin deposition disease (Randall type). Relationship with structural abnormalities of immunoglobulin chains. Kidney Int 1994: 46: 965-972.
Aucouturier P, Khamlichi AA. Touchard G. Justrabo E. Cogné M. Chauffert B. Martin F. and Preud 'Homme JL. Brief report: Heavy-chain deposition disease. N Engl J Med 1993: 329. nº 19.
Buxbaum JN, Chuba JV. Hellman GC. Solomon A. and Gallo GR. Monoclonal immunoglobulin deposition disease: Light chain and light and heavy chain deposition diseases and their relation to light chain amyloidosis. Ann lntern Med 1990: 112: 455-464.
Solomon A. and Weiss DT. Ominous consequences of immuno globulin deposition. N Engl J Med 1993: 329: 1422.1423.
Pelletier G. Fabre M. Attali P, Ldouch-Badr A. Ink O. Martin E. and Etienne JP. Light chain deposition disease presenting with hepatomegaly: na association with amyloid-like fibrils. Postgrad Med J 1988: 64: 804-808.
Mignon F, Morel-Maroger L. Cerf M. Preud'Homme JL. & Ri chet G. Depots hépatiques et rénaux de chaines légeres kappa révélateurs d 'une dysglobulinémie. Nephrologie 1980: 1: 167-170.
Hoffman-Guilaine C. Nochy D, Tricottet V. Mallet L. Bariety J. & Camilleri JP. La maladie des dépots de chaines légeres: une entité anatomoclinique. Ann Pathol 1984: 4: 105-113.
Additional Files
Published
How to Cite
Issue
Section
License
This work is licensed under a Creative Commons Attribution 4.0 International License.
Copyright (c) 2023 Medicina Interna