Primary Amyloidosis Associated with Gaucher's Disease

Abstract
Elevations in serum immunoglobulins [Ig], frequently monoclonal in nature, are known to occur in patients with the adult form of Gaucher''s disease. Amyloidosis is described in a 46 yr 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 yr 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 Ig present has specific affinity for glucosyl ceramide.

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