Life-threatening splenic hemorrhage in two patients with Gaucher disease

Massive splenomegaly is a frequent finding in patients with Gaucher disease , the most common of the sphingolipidoses. Even so, the risk for splenic ru pture and intracapsular hemorrhage has not been emphasized due to the rarit y of this occurrence and the fibrotic, rubbery consistency of splenic tissu e In these patients. We report two adult patients with type 1 Gaucher disea se who suffered life-threatening splenic bleeds that were not acutely diagn osed. Both patients ultimately required emergent splenectomies. Factors com plicating the diagnosis of splenic hemorrhage in patients with Gaucher dise ase are discussed. Am. J, Hematol, 64:140-142 2000, Published 2000 Wiley-Li ss, Inc.dagger.