Article

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

Internal Medicine Department, Faculty of Medicine, Gazi University, 40, Sok. 7/7 Burcak Apt., Bahcelievler, 06500, Ankara, Turkey.
International journal of hematology (impact factor: 1.17). 12/2008; 89(1):58-62. DOI:10.1007/s12185-008-0192-2
Source: PubMed

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.

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  • Article: Rosai-Dorfman Disease with nodal and extranodal involvements: A case report.
    Mehri Najafi-Sani, Hossein Saneian, Fatemeh Mahjoub
    [show abstract] [hide abstract]
    ABSTRACT: Rosai-Dorfman disease (RDD) is a rare lymphoproliferative disorder with nodal and extranodal involvements. Here we report a case of RDD in a 15-year-old female who presented with epigastric pain, fatigue, Raynaud phenomenon in fingers, submandibular lymphadenopathy, proptosis, hepatosplenomegaly, and round shape painless patches on the extensor surfaces. Histological examination of the submandibular lymph nodes and skin biopsy demonstrated evidences of RDD. Patient was treated with prednisone and thereafter, with azathioprine. After one year, prednisone was discontinued and all of the symptoms and signs, except proptosis, were resolved. This report highlights the extranodal manifestations of RDD. The presentation, differential diagnosis, and treatment are discussed.
    Journal of research in medical sciences 09/2011; 16(9):1251-6. · 0.46 Impact Factor
Data provided are for informational purposes only. Although carefully collected, accuracy cannot be guaranteed. The impact factor represents a rough estimation of the journal's impact factor and does not reflect the actual current impact factor. Publisher conditions are provided by RoMEO. Differing provisions from the publisher's actual policy or licence agreement may be applicable.

Keywords

2-chlorodeoxyadenosine
 
36-year-old man
 
bone involvement
 
effective treatment
 
extensive disease
 
follow-up
 
inguinal lymphadenopathy
 
lymph node biopsy
 
mass lesion
 
massive lymphadenopathy
 
occasional extranodal involvement
 
painless lymphadenopathy
 
positron emission tomography
 
rare lymphoproliferative disorder
 
RDD
 
Rosai-Dorfman disease
 
sinus histiocytosis
 
spinal cord compression
 
steroid therapy
 
T6-7 vertebral level