title: Bare lymphocyte syndrome: imaging findings in an adult.
abstract: Bare lymphocyte syndrome (BLS) is a rare primary immune disorder characterized by defective expression of human leukocyte antigen (HLA) on lymphocytes, often resulting in extensive and recurrent multi-organ infections. We describe a previously undiagnosed case of an adult woman who presented with radiological findings of severe bronchiectases, near-total granulomatous destruction of facial bones, and osteomyelitis. Diagnosis of BLS should be considered when evaluating children with unexplained bronchiectases or adults with long history of chronic multi-organ infections.
authors.affiliation: Department of Radiology, Universitair Ziekenhuis Antwerpen Wilrijkstraat 10 2650 Edegem, Belgium.
authors.forename: A
authors.initials: A
authors.lastname: Bernaerts
authors.forename: J E
authors.initials: JE
authors.lastname: Vandevenne
authors.forename: J
authors.initials: J
authors.lastname: Lambert
authors.forename: L S
authors.initials: LS
authors.lastname: De Clerck
authors.forename: A M
authors.initials: AM
authors.lastname: De Schepper
mesh_terms: D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D016511:Severe Combined Immunodeficiency / Q000000981:diagnostic imaging* / Q000473:pathology*; D014057:Tomography, X-Ray Computed