title: Response of pure red cell aplasia to cyclophosphamide after failure of mycofenolate mofetil in a patient with polyglandular syndrome type I.
abstract: A 26-year-old female with the classic major and minor components of autoimmune polyglandular syndrome type 1 was diagnosed as having pure red cell aplasia. Treatment with 1.5 g/d mycofenolate mofetil for 3 months failed to restore erythroid production. Treatment with cyclosporine A produced a good partial response but led to renal toxicity and was therefore substituted with cyclophosphamide, which had a good partial effect and lasted for 18 months. The relapse of anemia was not observed during the 6-month follow-up period after the cessation of treatment.
authors.affiliation: *Endocrinology Research Centre, Institute of Pediatric Endocrinology †Federal Research Center for Pediatric Hematology, Oncology, and Immunology, Moscow, Russian Federation.
authors.forename: Elizaveta M
authors.initials: EM
authors.lastname: Orlova
authors.forename: Maria A
authors.initials: MA
authors.lastname: Kareva
authors.forename: Maria A
authors.initials: MA
authors.lastname: Melikyan
authors.forename: Elena
authors.initials: E
authors.lastname: Boyakova
authors.forename: Valentina A
authors.initials: VA
authors.lastname: Peterkova
authors.forename: Alexey A
authors.initials: AA
authors.lastname: Maschan
mesh_terms: D000328:Adult; D003520:Cyclophosphamide / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D009154:Mutation; D016884:Polyendocrinopathies, Autoimmune / Q000150:complications* / Q000235:genetics / Q000503:physiopathology; D012010:Red-Cell Aplasia, Pure / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D014157:Transcription Factors / Q000235:genetics; D000096927:AIRE Protein
chemical_list: D003520:Cyclophosphamide; D007166:Immunosuppressive Agents; D014157:Transcription Factors; D000096927:AIRE Protein