title: 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.affiliation: Internal Medicine Department, Faculty of Medicine, Gazi University, 40, Sok. 7/7 Burcak Apt., Bahcelievler, 06500, Ankara, Turkey. ceylakonca@hotmail.com.
authors.forename: Ceyla
authors.initials: C
authors.lastname: Konca
authors.affiliation: Division of Hematology, Internal Medicine Department, Faculty of Medicine, Gazi University, 40, Sok. 7/7 Burcak Apt., Bahcelievler, 06500, Ankara, Turkey.
authors.forename: Zübeyde N
authors.initials: ZN
authors.lastname: Özkurt
authors.affiliation: Internal Medicine Department, Faculty of Medicine, Gazi University, 40, Sok. 7/7 Burcak Apt., Bahcelievler, 06500, Ankara, Turkey.
authors.forename: Müge
authors.initials: M
authors.lastname: Deger
authors.affiliation: Division of Hematology, Internal Medicine Department, Faculty of Medicine, Gazi University, 40, Sok. 7/7 Burcak Apt., Bahcelievler, 06500, Ankara, Turkey.
authors.forename: Zeynep
authors.initials: Z
authors.lastname: Akı
authors.affiliation: Division of Hematology, Internal Medicine Department, Faculty of Medicine, Gazi University, 40, Sok. 7/7 Burcak Apt., Bahcelievler, 06500, Ankara, Turkey.
authors.forename: Münci
authors.initials: M
authors.lastname: Yağcı
mesh_terms: D015762:2-Chloroadenosine / Q000031:analogs & derivatives* / Q000627:therapeutic use; D000328:Adult; D003839:Deoxyadenosines / Q000627:therapeutic use*; D015618:Histiocytosis, Sinus / Q000175:diagnosis / Q000188:drug therapy* / Q000473:pathology*; D006801:Humans; D008206:Lymphatic Diseases; D008228:Lymphoma, Non-Hodgkin; D008297:Male; D012074:Remission Induction; D013117:Spinal Cord Compression / Q000209:etiology
chemical_list: D003839:Deoxyadenosines; D015762:2-Chloroadenosine; C055994:2-chloro-3'-deoxyadenosine