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

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

Author information

1
Internal Medicine Department, Faculty of Medicine, Gazi University, 40, Sok. 7/7 Burcak Apt., Bahcelievler, 06500, Ankara, Turkey. ceylakonca@hotmail.com.
2
Division of Hematology, Internal Medicine Department, Faculty of Medicine, Gazi University, 40, Sok. 7/7 Burcak Apt., Bahcelievler, 06500, Ankara, Turkey.
3
Internal Medicine Department, Faculty of Medicine, Gazi University, 40, Sok. 7/7 Burcak Apt., Bahcelievler, 06500, Ankara, Turkey.

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.

PMID:
19020950
DOI:
10.1007/s12185-008-0192-2
[Indexed for MEDLINE]

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