PROGRESSIVE LIVER-FAILURE IN A PATIENT WITH ADULT NIEMANN-PICK DISEASE-ASSOCIATED WITH GENERALIZED AL AMYLOIDOSIS

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 amy loidosis of kappa type. Both diagnosis were made by biopsy, the NPD by bone marrow biopsy and fibroblast culture, the amyloidosis by liver b iopsy, Malignant non-Hodgkin lymphoma was not found. The patient, a 67 -year-old woman, died from hepatic coma subsequent to a progressive li ver failure. We discuss possible relations between the lysosomal stora ge disease and the development and rapid progression of amyloidosis.