Progressive liver failure in a patient with adult Niemann-Pick disease associated with generalized AL amyloidosis.
Virchows Arch
; 426(6): 635-9, 1995.
Article
en En
| MEDLINE
| ID: mdl-7655746
ABSTRACT
We report a case in which an adult form of Niemann-Pick disease (type B of NPD) was associated with a rapidly progressive generalized AL amyloidosis of kappa type. Both diagnosis were made by biopsy, the NPD by bone marrow biopsy and fibroblast culture, the amyloidosis by liver biopsy. Malignant non-Hodgkin lymphoma was not found. The patient, a 67-year-old woman, died from hepatic coma subsequent to a progressive liver failure. We discuss possible relations between the lysosomal storage disease and the development and rapid progression of amyloidosis.
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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Enfermedades de Niemann-Pick
/
Fallo Hepático
/
Amiloidosis
/
Hígado
Tipo de estudio:
Risk_factors_studies
Límite:
Aged
/
Female
/
Humans
Idioma:
En
Revista:
Virchows Arch
Asunto de la revista:
BIOLOGIA MOLECULAR
/
PATOLOGIA
Año:
1995
Tipo del documento:
Article
País de afiliación:
Alemania