Monoclonal gammopathies and their management in Gaucher disease type 1

 

Gaucher disease type 1 (GD1) is a rare autosomal recessive genetic disorder characterized by deficiency of the lysosomal enzyme glucocerebrosidase. Among the various clinical manifestations that have been well described for decades, a more recent finding has been the association of GD1 with increased risk of various malignancies, including the monoclonal gammopathy multiple myeloma. We present a brief review describing the relationship between GD1 and monoclonal gammopathies, as well as the modern standard in the monitoring and management of these conditions.