| Literature DB >> 25521082 |
Magy Abdelwahab1, Hadeel M SeifEldien.
Abstract
Gaucher disease (GD) is characterized by 3 clinical subtypes; type 1 GD (non-neuronopathic) and types 2 and 3 GD (acute and chronic neuronopathic forms, respectively). There are few reports of mesenteric and mediastinal lymphadenopathy, and none in type 1 GD or in African people. We report 8 Egyptian GD children (3 type 1 GD and 5 type 3 GD) who developed mesenteric and mediastinal lymphadenopathy despite receiving enzyme replacement therapy. Biopsy showed infiltration with Gaucher cells and no malignant changes. Pediatric physicians should be aware of possible lymphadenopathy associated with both types 1 and 3 GD as it can mimic malignancy.Entities:
Mesh:
Year: 2015 PMID: 25521082 DOI: 10.1097/MPH.0000000000000297
Source DB: PubMed Journal: J Pediatr Hematol Oncol ISSN: 1077-4114 Impact factor: 1.289