Hepatic amyloidosis with light chain deposition disease. A rare association.
Dig Liver Dis
; 32(9): 795-8, 2000 Dec.
Article
in En
| MEDLINE
| ID: mdl-11215561
ABSTRACT
Monoclonal immunoglobulin deposition diseases are due to pathological protein deposition in various tissues and organs. Protein deposits may be found in a single tissue or systemically and the organs most frequently involved are kidney, heart, peripheral nerves and the liver. Depending on the pattern of the deposits and the type of immunoglobulin, these diseases are distinguished as primary amyloidosis, light chain deposition disease. Differential diagnosis is made in tissue specimens microscopically by the identification of positive Congo red staining of the deposits, by immunohistochemical demonstration of proteins reacting with light chain (lambda or kappa) antisera or by recognition of fibrillar structures on electron microscopy. We report an unusual case of light chain deposition disease associated with amyloidosis, where hepatomegaly was the presenting manifestation and liver failure the cause of death, without any kidney involvement.
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Collection:
01-internacional
Database:
MEDLINE
Main subject:
Immunoglobulin Light Chains
/
Amyloidosis
/
Hypergammaglobulinemia
/
Liver Diseases
Type of study:
Diagnostic_studies
/
Etiology_studies
/
Prognostic_studies
/
Risk_factors_studies
Limits:
Humans
/
Male
/
Middle aged
Language:
En
Journal:
Dig Liver Dis
Journal subject:
GASTROENTEROLOGIA
Year:
2000
Document type:
Article
Affiliation country: