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


Konca C., Oezkurt Z. N. , Deger M., Aki Z., Yagci M.

INTERNATIONAL JOURNAL OF HEMATOLOGY, cilt.89, sa.1, ss.58-62, 2009 (SCI İndekslerine Giren Dergi) identifier identifier identifier

  • Cilt numarası: 89 Konu: 1
  • Basım Tarihi: 2009
  • Doi Numarası: 10.1007/s12185-008-0192-2
  • Dergi Adı: INTERNATIONAL JOURNAL OF HEMATOLOGY
  • Sayfa Sayıları: ss.58-62

Özet

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.