title: Lymphoproliferative disorder of the airway of an adolescent without immunodeficiency.
abstract: Epstein-Barr virus (EBV) is a ubiquitous viral pathogen in humans that has a unique ability to immortalize B-cells. In immunosuppressed individuals, EBV can produce non-neoplastic lymphoproliferative disorders involving various organs. We describe a case report of EBV-associated lymphoproliferative disorder in an immunocompetent 14-year-old male. The case provides a description of EBV-associated lymphoproliferation affecting the upper and lower respiratory tract. The massive submucosal infiltration of B-cells in the lingual tonsils, trachea, and bronchi produced near-complete airway obstruction resulting in tracheotomy. Neither surgical reduction of lingual tonsils nor treatment with steroids was of benefit. An extensive evaluation for immunodeficiency and neoplasia was normal. Treatment with rituximab, an anti-CD20 antibody, resulted in near-complete resolution of the infiltrative process, sufficient to allow decannulation. Rituximab is a treatment option for the rare occurrence of non-neoplastic, EBV-associated, lymphoproliferative disorders.
authors.affiliation: Division of Pediatric Pulmonary Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229, USA. Paul.Boesch@cchmc.org
authors.forename: R P
authors.initials: RP
authors.lastname: Boesch
authors.forename: M
authors.initials: M
authors.lastname: Daines
authors.forename: A
authors.initials: A
authors.lastname: Kaul
authors.forename: R
authors.initials: R
authors.lastname: Cotton
authors.forename: R
authors.initials: R
authors.lastname: Amin
mesh_terms: D000293:Adolescent; D000402:Airway Obstruction / Q000188:drug therapy / Q000209:etiology*; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D020031:Epstein-Barr Virus Infections / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D007121:Immunocompetence; D007155:Immunologic Factors / Q000627:therapeutic use; D008232:Lymphoproliferative Disorders / Q000175:diagnosis* / Q000188:drug therapy / Q000821:virology*; D008297:Male; D000069283:Rituximab
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D000069283:Rituximab