title: Mesenteric and Mediastinal Lymphadenopathy in Egyptian Children With Gaucher Disease Types 1 and 3 Treated With Enzyme Replacement Therapy.
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.
authors.affiliation: Departments of *Pediatric Hematology †Pediatric Radiology, Cairo University Pediatric Hospital, Cairo, Egypt.
authors.forename: Magy
authors.initials: M
authors.lastname: Abdelwahab
authors.forename: Hadeel M
authors.initials: HM
authors.lastname: SeifEldien
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D002648:Child; D004534:Egypt; D056947:Enzyme Replacement Therapy; D005260:Female; D005776:Gaucher Disease / Q000188:drug therapy* / Q000473:pathology*; D006801:Humans; D007223:Infant; D008206:Lymphatic Diseases / Q000209:etiology*; D008297:Male; D008482:Mediastinum / Q000473:pathology*; D008643:Mesentery / Q000473:pathology*