title: Progressive liver failure in a patient with adult Niemann-Pick disease associated with generalized AL amyloidosis.
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.
authors.affiliation: Pathologisches Institut der Universität, Bonn, Germany.
authors.forename: H
authors.initials: H
authors.lastname: Zhou
authors.forename: R P
authors.initials: RP
authors.lastname: Linke
authors.forename: H E
authors.initials: HE
authors.lastname: Schaefer
authors.forename: W
authors.initials: W
authors.lastname: Möbius
authors.forename: U
authors.initials: U
authors.lastname: Pfeifer
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000150:complications* / Q000473:pathology; D005260:Female; D006801:Humans; D008099:Liver / Q000473:pathology*; D017093:Liver Failure / Q000209:etiology* / Q000473:pathology; D008854:Microscopy, Electron; D009542:Niemann-Pick Diseases / Q000150:complications* / Q000473:pathology