Clinical lymphadenopathy is a less common manifestation of Gaucher's disease.
Risk factors for lymphadenopathy:
(1) severe GD1 phenotype, or GD3
(2) early onset Gaucher's disease (manifestation of more severe disease)
(3) status post splenectomy (usually associated with hepatosplenomegaly)
Clinical features:
(1) commonly affects mediastinal and mesenteric lymph nodes, but may affect any region
(2) may be initially diagnosed as malignant lymphoma or metastatic cancer
Complications:
(1) protein losing enteropathy (associated with weight loss, failure to thrive, diarrhea, other gastrointestinal symptoms, hypoproteinemia and peripheral edema)
(2) severe systemic involvement
