Bare lymphocyte syndrome: imaging findings in an adult.
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.
Bernaerts
A
A
Department of Radiology, Universitair Ziekenhuis Antwerpen Wilrijkstraat 10 2650 Edegem, Belgium.
Vandevenne
J E
JE
Lambert
J
J
De Clerck
L S
LS
De Schepper
A M
AM
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