Response of Pure Red Cell Aplasia to Cyclophosphamide After Failure of Mycofenolate Mofetil in a Patient With Polyglandular Syndrome Type I

• DOI: 10.1097/mph.0b013e3182755c52
• Publication Date: 2013-01-18T08:11:12Z
• Authors: Elizaveta M. Orlova; Maria A. Kareva; Maria A. Melikyan; Elena Boyakova; Valentina A. Pete...; Alexey A. Maschan
• Keywords: Adult; 0301 basic medicine; AIRE Protein; Red-Cell Aplasia, Pure; 3. Good health; 03 medical and health sciences; Mutation; Humans; Female; Polyendocrinopathies, Autoimmune; Cyclophosphamide; Immunosuppressive Agents; Transcription Factors

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.