SAMIR M. HANASH, M.D.; DONALD L RUCKNAGEL, M.D.; KATHLEEN P. HEIDELBERGER, M.D.; NORMAN S. RADIN, Ph.D.
Elevations in serum immunoglobulins, frequently monoclonal in nature, are known to occur in patients with the adult form of Gaucher's disease. We describe amyloidosis in a 46-year-old woman of Italian ancestry with Gaucher's disease, who also had 3100 mg/dl of a monoclonal IgA. She died of restrictive cardiac disease. A 50-year-old sister, also with Gaucher's disease, had 1300 mg/dl of polyclonal IgM but no evidence of amyloidosis. A glucosyl sphingosine-Sepharose affinity column provided no evidence that the large amount of immunoglobulin present has specific affinity for glucosyl ceramide. This, to our knowledge, is the first report of amyloidosis complicating Gaucher's disease.
HANASH SM, RUCKNAGEL DL, HEIDELBERGER KP, et al. Primary Amyloidosis Associated with Gaucher's Disease. Ann Intern Med. 1978;89:639–641. doi: https://doi.org/10.7326/0003-4819-89-5-639
Download citation file:
Published: Ann Intern Med. 1978;89(5_Part_1):639-641.
Results provided by:
Copyright © 2020 American College of Physicians. All Rights Reserved.
Print ISSN: 0003-4819 | Online ISSN: 1539-3704
Conditions of Use