Gauchers disease

Several case reports have long suspected an association between Gaucher's disease and gammopathy.34-39 There is a reported incidence of 20% polyclonal gam-mopathy in this disease with an unclear percentage of monoclonal abnormalities. It is suspected that lipid deposition in Gaucher's disease results in chronic stimulation of the humoral immune system with production of polyclonal immunoglobulins that under unclear circumstances become monoclonal with development of MGUS and/or MM.40 The use of enzyme therapy to control Gaucher's disease decreased polyclonal immunoglobulin levels but had no effect on monoclonal gammopathy. The lack of correlation between disease activity and immunoglobulin levels means that the enzyme-sensitive process affecting lipid metabolism is not directly linked to immunoglobulin deregulation.41

0 0

Post a comment