ISSN:
1432-2307
Keywords:
Adult Niemann-Pick disease
;
Generalized AL-amyloidosis
;
Progressive liver failure
;
Fibroblast culture
;
Immunohistochemistry
;
Electron microscopy
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
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.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00192120
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