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Splenectomy in Gaucher Disease: New Management Dilemmas 722

Gaucher disease, the inherited deficiency of lysosomal glucocerebrosidase, is currently managed with enzyme replacement therapy using a mannose-terminated form of human glucocerebrosidase. Although most patients with type 1 Gaucher disease respond to this therapy, we describe an Ashkenazi patient with type 1 Gaucher disease with massive hepatosplenomegaly, anemia, and thrombocytopenia, who despite two years of aggressive high dose enzyme replacement therapy with Alglucerase, ultimately required a splenectomy for effective management. The removed spleen weighed 6.5kg and had nodular appearing external surfaces and focal subcapsular infarcts. Serial sections showed rubbery and focally fibrotic surfaces. This correlated with his presurgical axial T2-weighted MRI, which demonstrated multiple low signal intensity nodules not seen on T1-weighted views. Postoperatively the patient has remained stable with normal blood and platelet counts.