Lymphoproliferative disorder of the airway of an adolescent without immunodeficiency

Int J Pediatr Otorhinolaryngol. 2005 Nov;69(11):1591-4. doi: 10.1016/j.ijporl.2005.04.028. Epub 2005 Jun 15.

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.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Airway Obstruction / drug therapy
  • Airway Obstruction / etiology*
  • Antibodies, Monoclonal / therapeutic use
  • Antibodies, Monoclonal, Murine-Derived
  • Epstein-Barr Virus Infections / diagnosis*
  • Epstein-Barr Virus Infections / drug therapy
  • Humans
  • Immunocompetence*
  • Immunologic Factors / therapeutic use
  • Lymphoproliferative Disorders / diagnosis*
  • Lymphoproliferative Disorders / drug therapy
  • Lymphoproliferative Disorders / virology*
  • Male
  • Rituximab

Substances

  • Antibodies, Monoclonal
  • Antibodies, Monoclonal, Murine-Derived
  • Immunologic Factors
  • Rituximab