Zhou H, Linke R P, Schaefer H E, Möbius W, Pfeifer U
Pathologisches Institut der Universität, Bonn, Germany.
Virchows Arch. 1995;426(6):635-9. doi: 10.1007/BF00192120.
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.
