AH amyloidosis associated with lymphoplasmacytic lymphoma secreting a monoclonal gamma heavy chain carrying an unusual truncated D segment.
Am J Kidney Dis
; 47(5): 908-14, 2006 May.
Article
em En
| MEDLINE
| ID: mdl-16632032
ABSTRACT
To date, the presence of amyloidosis associated with immunoglobulin heavy chain (AH amyloidosis) was reported in only 7 cases. Although AH amyloidosis is caused mainly by plasma cell dyscrasia, as in AL amyloidosis, we report a 61-year-old patient who presented with nephrotic syndrome caused by AH amyloidosis associated with lymphoplasmacytic lymphoma. Biochemical and molecular analyses of the deposited amyloid fibrils and heavy-chain genes of lymphocytes showed that proliferative lymphoma cells produced a gamma heavy chain, not a mu heavy chain, which carried an unusual truncated diversity (D) segment of the variable region. Our results indicate that production of the abnormal heavy chain caused by the partially deleted D segment gene is responsible for gamma heavy-chain-related amyloid fibril formation in this patient.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Leucemia Linfocítica Crônica de Células B
/
Cadeias gama de Imunoglobulina
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Cadeias Pesadas de Imunoglobulinas
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Amiloidose
Tipo de estudo:
Risk_factors_studies
Limite:
Female
/
Humans
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Middle aged
Idioma:
En
Ano de publicação:
2006
Tipo de documento:
Article