Extranodal multifocal Rosai-Dorfman disease: response to 2-chlorodeoxyadenosine treatment

Int J Hematol. 2009 Jan;89(1):58-62. doi: 10.1007/s12185-008-0192-2. Epub 2008 Nov 20.


Rosai-Dorfman disease (RDD) or "sinus histiocytosis with massive lymphadenopathy" is a rare lymphoproliferative disorder of unknown etiology. The disease usually presents with painless lymphadenopathy with occasional extranodal involvement in various organs. We report a case of a 36-year-old man with a history of non-Hodgkin lymphoma (NHL), who recently presented with inguinal lymphadenopathy. Following the diagnosis of RDD on lymph node biopsy, he developed symptoms of spinal cord compression due to a mass lesion discovered at T6-7 vertebral level. 18F-Fluorodeoxyglucose (18FDG) positron emission tomography (PET-CT) revealed extensive disease with lung, renal and bone involvement. The patient received a short course of steroid therapy for cord compression findings and 2-chlorodeoxyadenosine (2-CdA) treatment was initiated for long-term disease control. He had a dramatic sustained response to treatment with six courses of 2-CdA. These results suggest that 2-CdA can be an effective treatment of choice and positron emission tomography with 18FDG can be used for determining the extent of disease and for follow-up in RDD.

Publication types

  • Case Reports

MeSH terms

  • 2-Chloroadenosine / analogs & derivatives*
  • 2-Chloroadenosine / therapeutic use
  • Adult
  • Deoxyadenosines / therapeutic use*
  • Histiocytosis, Sinus / diagnosis
  • Histiocytosis, Sinus / drug therapy*
  • Histiocytosis, Sinus / pathology*
  • Humans
  • Lymphatic Diseases
  • Lymphoma, Non-Hodgkin
  • Male
  • Remission Induction
  • Spinal Cord Compression / etiology


  • Deoxyadenosines
  • 2-Chloroadenosine
  • 2-chloro-3'-deoxyadenosine