Unusual clinical manifestation of virus-associated hemophagocytic syndrome

Pathol Res Pract. 2003;199(11):755-9. doi: 10.1078/0344-0338-00493.

Abstract

A 22-year-old man presented with bilateral painless cervical lymphadenomegaly, difficulties in nasal breathing and bilateral conductive hearing loss. Rhinoscopy and computer tomography disclosed mucosal polyps in the nasal cavity and a polypoid soft mass almost completely filling the whole nasal cavity and the paranasal sinuses. Thoracic and abdominal computer tomography showed mild hepatosplenomegaly and a solitary round lesion in the right lung. No fever or abnormal laboratory parameters were detected. The biopsy from the nasal polypoid lesions and from the cervical lymph nodes showed extensive proliferation of histologically benign erythrophagocytic histiocytes. The diagnosis of virus (Epstein-Barr virus)-associated hemophagocytic histiocytosis was confirmed by immunohistochemical reactions, by polymerase chain reaction, and by Epstein-Barr-Encodes (Early)-RNA in situ hybridization. This case illustrates an unusual clinical manifestation of virus-associated hemophagocytic histiocytosis presenting as mucosal polyps of the upper respiratory tract.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Epstein-Barr Virus Infections / complications*
  • Epstein-Barr Virus Infections / virology
  • Genome, Viral
  • Herpesvirus 4, Human / genetics
  • Histiocytosis, Non-Langerhans-Cell / complications
  • Histiocytosis, Non-Langerhans-Cell / metabolism
  • Histiocytosis, Non-Langerhans-Cell / pathology*
  • Histiocytosis, Non-Langerhans-Cell / virology*
  • Humans
  • Immunohistochemistry
  • In Situ Hybridization
  • Male
  • Nasal Polyps / etiology*
  • RNA, Messenger / metabolism
  • RNA, Viral / metabolism

Substances

  • RNA, Messenger
  • RNA, Viral