Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) in a patient with elevated Epstein-Barr virus titers.
Sinus histiocytosis with massive lymphadenopathy (SHML) is a newly recognized disorder. The etiology of this disease is unknown. An exaggerated response to an offending agent such as the Epstein-Barr virus or Klebsiella bacteria has been postulated. Its course is usually benign. Cervical adenopathy is seen in 97% of the patients, while 30% of patients have nodal involvement in other sites, and 30% have extranodal involvement. There is a 7% mortality rate that occurs primarily in patients with immunologic defects. Corticosteroids ameliorate the constitutional symptoms, but cyclophosphamide appears to have the most beneficial effect. This article presents the case of a patient with SHML who demonstrated elevated Epstein-Barr virus titers.
Harley
E H
EH
Department of Otolaryngology, Naval Hospital, San Diego, California.
D004854:Herpesvirus 4, Human / Q000302:isolation & purification*; D015618:Histiocytosis, Sinus / Q000097:blood / Q000382:microbiology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009333:Neck