title: Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): response to methotrexate and mercaptopurine.
abstract: We report a 3-year-old girl presenting with bilateral cervical lymph node enlargement persisting for > 3 months. Leukocytosis, elevated erythrocyte sedimentation rate, a marked hypergammaglobulinemia, and a moderate hepatosplenomegaly were also found. The diagnosis of sinushistiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease, was established histologically by the demonstration of characteristic sinushistiocytosis with lymphocytophagocytosis. Treatment was started with high dose steroids, and a decline of lymph node size and a normalization of laboratory parameters occurred. However, when steroids were tapered, lymph node size rapidly reincreased. Chemotherapeutic treatment was started using etoposide, which was completely ineffective. Therefore, treatment was changed to a combinatory low dose methotrexate therapy and 6-mercaptopurine for 4 months. Whereas a prompt and complete remission was reached, single 6-mercaptopurine therapy was maintained and treatment has been discontinued after a total of 2 years. The child has remained healthy for 7 years. This case would recommend the use of methotrexate and 6-mercaptopurine for treatment of complicated SHML.
authors.affiliation: Department of Pediatrics, University of Duesseldorf, Germany.
authors.forename: G
authors.initials: G
authors.lastname: Horneff
authors.forename: H
authors.initials: H
authors.lastname: Jürgens
authors.forename: W
authors.initials: W
authors.lastname: Hort
authors.forename: D
authors.initials: D
authors.lastname: Karitzky
authors.forename: U
authors.initials: U
authors.lastname: Göbel
mesh_terms: D000963:Antimetabolites / Q000008:administration & dosage*; D002675:Child, Preschool; D004359:Drug Therapy, Combination; D005260:Female; D015618:Histiocytosis, Sinus / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D008198:Lymph Nodes / Q000473:pathology; D015122:Mercaptopurine / Q000008:administration & dosage*; D008727:Methotrexate / Q000008:administration & dosage*
chemical_list: D000963:Antimetabolites; D015122:Mercaptopurine; D008727:Methotrexate