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

Abstract

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.

• Authors: Ceyla Konca, Zübeyde N Özkurt, Müge Deger, Zeynep Akı, Münci Yağcı
• Journal: International journal of hematology (Int J Hematol) Vol. 89 Issue 1 Pg. 58-62 (Jan 2009) ISSN: 1865-3774 [Electronic] Japan
• PMID: 19020950 (Publication Type: Case Reports, Journal Article)

Chemical References

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

Topics

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