EFFICACY OF INTERFERON
a-2b IN ROSAI-DORFMAN DISEASE
Gonz¨¢lez-Vite M, Novales-Castro X, Ayala-Zavala M, Torres-Falf¨¢n MD, Cruz-Olivo F
Hospital General Centro M¨¦dico Nacional La Raza, IMSS, M¨¦xico
Rosai-Dorfman disease or
sinus histiocytosis with massive limphadenopathy is a well-recognized
disorder whose typical clinical characteristics included massive painless
cervical, axilary, inguinal and mediastinal limphadenopathy. Extranodal
manifestation of the disease is well documented. Its pathogenesis is
uncertain and is thought to be a disorder of immune regulation or a
response to a presumed infection. The diagnosis is currently definite by
histological features.
Case report: We observed a 13 years old male
adolescent with progressive right submandibular limphadenopathy. A chest
roentgenogram and complete blood cell count were normal. However the
erythrocyte sedimentation rate was prolonged and quantitative serum
inmunoglobulins levels were elevated. Then, a lymph node biopsy was
performed. The affected lymph node showed a distinct histological
morphology with dilated sinus, which was filled with numerous histiocytes
showing large clear cytoplasm and a marked lymphocytophagocytosis. With
these histological features the diagnosis was confirmed. During the
following months limphadenopathy continued to progress with additional
involvement of cervical, inguinal and mediastinal lymphs nodes. He received
prednisone with transient result. Then, the patient received 3 miu of
interferon ¦Á-2b two times a week for
10 months. His adenopathy was reduced by about 90% by volume.
Discussion: Rosai-Dorfman disease is an uncommon
proliferative disorder of histiocytes. Most patients do not require
specific therapy and can expect spontaneous regression. At present,
treatment using interferon may be indicated for patients with persistent or
progressive disease. We started an interferon treatment with good results
until now.