Expansion of hematogones in a patient with gaucher disease
β Scribed by D'Arena, Giovanni ;Bisceglia, Michele ;Ladogana, Saverio ;Carella, Angelo Michele ;Carotenuto, Mario ;Paolucci, Paolo
- Publisher
- John Wiley and Sons
- Year
- 2001
- Tongue
- English
- Weight
- 129 KB
- Volume
- 36
- Category
- Article
- ISSN
- 0098-1532
- DOI
- 10.1002/mpo.1147
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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 tissu
## Communicated by Mark Paalman Gaucher disease, the most common lysosomal storage disorder, results from the inherited deficiency of the enzyme glucocerebrosidase. Three clinical types are recognized: type 1, nonneuronopathic; type 2, acute neuronopathic; and type 3, subacute neuronopathic. Type 2