title: Rosai-Dorfman disease with diffuse gastrointestinal involvement.
abstract: Rosai-Dorfman disease, formerly known as 'sinus histiocytosis with massive lymphadenopathy', is a rare self-limiting histiocytic proliferative disorder typically presenting early in life with cervical lymphadenopathy and nonspecific systemic symptoms. Although it is usually a nodal disease, extranodal lesions may be encountered in some cases. The gastrointestinal tract is uncommonly affected in Rosai-Dorfman disease and its diagnosis depends on clinical suspicion and careful histopathological examination of biopsy samples taken from involved gastrointestinal segments. Here, we report a case of atypical Rosai-Dorfman disease with systemic symptoms and diffuse gastrointestinal involvement that led to a diagnostic and therapeutic challenge.
authors.affiliation: Department of Gastroenterohepatology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.
authors.forename: Bulent
authors.initials: B
authors.lastname: Baran
authors.forename: Cetin
authors.initials: C
authors.lastname: Karaca
authors.forename: Ozlem M
authors.initials: OM
authors.lastname: Soyer
authors.forename: Ipek
authors.initials: I
authors.lastname: Yonal
authors.forename: Aysun D
authors.initials: AD
authors.lastname: Isiklar
authors.forename: Ramazan
authors.initials: R
authors.lastname: Cakmak
authors.forename: Meliha
authors.initials: M
authors.lastname: Nalcaci
authors.forename: Oner
authors.initials: O
authors.lastname: Dogan
authors.forename: Sabahattin
authors.initials: S
authors.lastname: Kaymakoglu
mesh_terms: D000368:Aged; D001706:Biopsy; D004359:Drug Therapy, Combination; D016099:Endoscopy, Gastrointestinal; D019160:Endosonography; D017809:Fatal Outcome; D005765:Gastrointestinal Agents / Q000627:therapeutic use; D005767:Gastrointestinal Diseases / Q000175:diagnosis / Q000628:therapy; D005770:Gastrointestinal Neoplasms / Q000175:diagnosis; D015618:Histiocytosis, Sinus / Q000175:diagnosis / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D011237:Predictive Value of Tests; D016896:Treatment Outcome
chemical_list: D005765:Gastrointestinal Agents; D007166:Immunosuppressive Agents