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 rupture and intracapsular hemorrhage has not been emphasized due to the rarity of this occurrence and the fibrotic, rubbery consistency of splenic tissue in these patients. We report two adult patients with type 1 Gaucher disease who suffered life-threatening splenic bleeds that were not acutely diagnosed. Both patients ultimately required emergent splenectomies. Factors complicating the diagnosis of splenic hemorrhage in patients with Gaucher disease are discussed. Published 2000 Wiley-Liss, Inc.
Stone
D L
DL
Clinical Neuroscience Branch, National Institute of Mental Health, National Institutes of Health, Bethesda, Maryland, USA.
Ginns
E I
EI
Krasnewich
D
D
Sidransky
E
E
D000328:Adult; D016638:Critical Illness; D004632:Emergency Medical Services; D005776:Gaucher Disease / Q000150:complications*; D006470:Hemorrhage / Q000150:complications* / Q000000981:diagnostic imaging / Q000601:surgery; D006801:Humans; D008297:Male; D008875:Middle Aged; D013156:Splenectomy; D013158:Splenic Diseases / Q000150:complications* / Q000000981:diagnostic imaging / Q000601:surgery; D014057:Tomography, X-Ray Computed